MHC molecules are peptide receptors present on the surface of nearly all cells in the body. Their function in the individual is to bind peptide fragments of larger degraded antigen systems, and these MHC peptide fragments are the "antigen" that that triggers T lymphocytes. This follows from the fact that T cells with receptors of high avidity for MHC/peptide are eliminated in the thymus (tolerance to self molecules); T cells with no avidity for MHC/peptide fail to survive. Comparison of MHC class I and MHC class II pathways CharacteristicMHC IMHC II Subunit compositionpolymorphic heavy ( chain + ß2 microglobulinpolymorphic ( and ß chains Tissue Expressionall nucleated cellslimited to B cells, M(, DC, thymic epithelium TCR engagedCD8+ T cells CD4+ T cells Peptides presented ~9 aa16-24 aa Allele specific motifs well defined: cathepsins peptide loading compartmentERMIIC (novel endosome) MHC transport to assembly compartment ER, constitutive invariant chain (Ii) acts as chaperone to carry MHC II to MIIC compartment Peptide transport to MHCvia transporter of antigenic peptides (TAP)? not necessary; MHC and peptides in same vesicles Peptide loading facilitated by MHC association with TAP, calnexin?

Acute pancreatitis is a common disorder that results from acute inflamatory injury of the pancreas.The clinical picture ranges from mild disease to multiorgan failure and sepsis, specially in the severe necrotizing or hemorragic forms of disease. And although the diagnosis is not a major concern, the treatment has been largely empirical since the mechanims by which the well recognized etiological factors lead to the development of the disease remains to be stablished.In others words, the actual sequence of events resulting in disease causation and evolution are not well known and, as a consequence, the results of the current therapeutic approaches are disappointing. For this reason, strong efforts have been made to elucidate the pathophysiology of acute pancreatitis, since this will lead to the development of better remedies and perhaps to the early recognition of local and systemic complications, with a lower mortality rate and better outcome to our patients. The main goal of this article is to summarize the current concepts about the etiology of acute pancreatitis, and how these agressive factors lead to pancreatic tissue damage and systemic involvement that is typical of this disease. Traumatic causes of disease include blunt abdominal trauma, iatrogenic pancreatitis resulting from ERCP, specially in the case of endoscopical sphincterotomy, and after thoracic surgery, mainly after cardiopulmonary bypass, risk factors being hypoperfusion of pancreatic tissue and hypercalcemia due to the injection of calcium chloride. Since many of the patients tought to have alcoholic acute pancreatitis have histophatological features of cronic pancreatitis and the majority of then will develop chronic pancreatitis after the acute episode, still many experts do not believe in the existence of this entity.

The distribution of cholesterol in mammalian cells is tightly controlled (reviewed in Liscum and Underwood, 1995). At steady state, most cellular cholesterol resides in the plasma membrane, where it plays an essential role in maintaining membrane fluidity. The factors that maintain high cholesterol levels in the plasma membrane are not clear. Plasma membrane cholesterol appears to be in dynamic equilibrium with intracellular membranes. Natalie Jacobs's work suggests that movement of cholesterol to the plasma membrane both from the endoplasmic reticulum and through lysosomes is normal, while movement of plasma membrane cholesterol to the endoplasmic reticulum is defective. Natalie Jacobs's work suggests that movement of cholesterol to the plasma membrane both from the endoplasmic reticulum and through lysosomes is normal, while movement of plasma membrane cholesterol to the endoplasmic reticulum is defective.

Audio Tapes of my November 30th, 1996 two hour live radio interview done on the Laura Lee show are available at the Laura Lee site.This tape includes about 45 minutes of interview with two other users of the device. An electrode device is not the device that produced the results Dr. One should think of this device as behaving as a cross between a TENS unit,a short wave diathermy,a micro current, and ultra an sound therapeutic device for comparison. Which is part of why the audio frequency from the square wave generator is varied to produce different physiologic effects.The book and video tape has pictures of micro organisms which were destroyed by the device, through the air,with no direct connections between the device and the micro organism from 4 to 5 feet away. " Resonant Frequency Therapy - Building the Rife Beam Ray Device " contains 23 short illustrated Chapters covering; the development, full construction, and operation of the device. The video tape has a section devoted to assembling all the parts into a completed device and then operating the device.

My research involves the use of x-ray crystallography, organic chemistry, and molecular biology to study the structure and function of proteins involved in disease and cell signaling. It has a number of interesting biological properties, including antiviral activity, inhibition of protein synthesis, initiation of apoptosis (programmed cell death), and ability to arrest the cell cycle.  It has potential as an anticancer and immunosuppressive drug, and is currently in Phase II clinical trials with the National Cancer Institute against a variety of human cancers and leukemias. PPT was originally isolated for its ability to remove palmitate from palmitoylated H-Ras; however, recent results indicate that PPT is targeted to the lysosome and therefore Ras may not be the the enzyme's natural target. Very little is known about PPT, and there is no structural information. We are determining the crystal structure of PPT with didemnin B bound to gain insight into the structure and mechanism of PPT and determine the mode of binding and inhibition by didemnin B. This information will be useful in learning more about the role of PPT in cellular signaling, and how didemnin B can disrupt the cell cycle. I am also interested in the structure and function of modular protein domains involved in cell signaling events and molecular recognition, particularly the PH (pleckstrin homology) and DH (Dbl homology) domains.

Still in its very early stages, Dr Frances Platt and Dr Terry Butters, explain how it works: For the past few years we have been studying the activities of a substance called NB-DNJ, trying to understand why it inhibits the replication of certain viruses. New Activity of the Drug, NB-DNJ In the course of our experiments, we made the unexpected observation that NB-DNJ inhibits an important cellular enzyme. The second is gene therapy, which aims to introduce a copy of the normal gene into the patient which will lead to the production of the normal enzyme and correct the enzyme deficiency. NB-DNJ and the Prevention of Lysosomal Storage Over the last two years we have been investigating the therapeutic potential of NB-DNJ in three separate ways. We are now studying cells derived from patients suffering from Gauchers disease and Tay-Sachs disease (kindly provided by Dr Bryan Winchester, Institute of Child Health, London) to see if we can prevent accumulation of glycolipids with NB-DNJ treatment, and to see if NB-DNJ treated cells can degrade the material which has already been stored within the lysosome. Identification of a Second Drug We have recently identified a second, more selective drug called NB-DGJ (which is very similar to NB-DNJ).

Membrane traffic to the lysosome/vacuole - Membrane traffic to the lysosome/vacuole /smj/books/ed/science/biomol/guideboo/spath/part5/a /smj/books/ed/science/biomol/guideboo/spath/part5/b We welcome comment or feedback on any of our pages. These pages are maintained by www-admin@oup.co.uk, please mail us if you have a problem using this service.

The endocytic vesicle or endosome contains the microbe and is soon joined by a lysosome (pink) to form an endolysosome, the "digestive body" of the cell. Cells consume "food and water" from extracellular spaces. Proteins (including microbes) that enter cells must be "digested" or broken into their component amino acids. In the above example, a cell called a macrophage or "Big Eater" encounters a microbe it "eats" it. In a process called phagocytosis, the cell extends finger/veil-like extensions of its cytoplasmic membane and engulfs the microbe. Confusion might result from the terms endosome, phagolysosome, phagolysosome, primary and secondary lysosomes, coated and uncoated vesicles, and curl vesicles.

e.g. Nike, free delivery, roses HELPFUL HINTS To do a search, you can fill in almost any combination of fields in this form (note that all fields are optional). To search for businesses within a certain distance of a location that you provide, press the "Search by Distance. Be sure to enter a state for the FASTEST search. Use commas to separate alternate search terms or locations. For example, you can enter "NY, MA, CT" in the state field to search for businesses in these three states.

We are not very successful in vaccinating against the following - A. Diphtheria and tetanus B. Measles and mumps C. Colds and viral flu D. Polio and chicken pox 16. The MHC on a macrophage is considered a class II antigen - it is involved in the immune response by - A. Presenting an epitope B. Synthesizing antibody C. Destruction of virus D. Destruction of aberrant cells 24. A T-cell travels about the human circulatory system and recognizes A. MHC class I (normal) B. MHC class II (normal) C. Altered MHC class II on macrophages D. All of the above 25. If an individual is effectively vaccinated against tetanus and later receives a second vaccination (booster shot) with an equivalent vaccine - the following would occur (relative to first shot) A. Shorter lag before circulating antibody produced in quantity B. More rapid production of antibody C. Longer plateau D. A and B only are correct E. A, B, and C are correct 29. A major respiratory disease that occurs in a high percentage of the population each year A. Anthrax and typhus B. Cholera and salmonellosis C. Gonorrhea and syphilis D. Colds and flu 33. Which of the following would be correct A. Animals make up about 50% of the biosphere B. Plants make up about 15% of the biosphere C. Microbes make up about 35% of the biosphere D. All are correct E. None are correct 63.

Protein sorting in Golgi default processing: constitutive secretion KDEL signal (go back to ER) - vessicles move back on MT

Structure/function studies of lysosomal enzymes using crystallography (in collaboration with Dr Mitchell Guss, Department of Biochemistry, University of Sydney) and affinity labelling with substrate analogues. Diagnosis of lysosomal storage disorders. Peroxisomal research encompassing fatty acid oxidation and ether lipid biosynthesis, transport mechanisms and pathogeneisis of peroxisomal disease Peroxisomal membrane transport of fatty acids Molecular biology of metachromatic leucodystrophy[Go Home][Laboratory Profile][Contact senior staff][Tests and Specimen requirements] [Sample preparation][Sample transport][Laboratory Experience][Charges for tests]

Significance of cell surface expression of lysosome-associated membrane proteins (LAMPs); 2. Lysosome-associated membrane proteins (LAMPs) are transmembrane glycoproteins which are densely expressed on lysosomes but are shuttled to the cell surface during cell activation. Their functional significance, both on lysosomes and at the cell surface, is unclear. High cell surface LAMP expression is associated with tumor cell lines which have a high metastatic potential, with lymphoid cells in patients with scleroderma and systemic lupus erythematosus, with IL8 stimulation in vitro, and with platelets that are activated in vitro and in vivo. Cell surface expression of lysosome-associated membrane proteins in scleroderma: Relationship of lamp2 to disease duration, anti-Sc170 antibodies, serum interleukin-8 and soluble interleukin-2 receptor levels. Cell surface expression of lysosome-associated membrane protein-2 (lamp2) and CD63 as markers for in vivo platelet activation in malignancy.

David C. Amberg, Ph.D. Dartmouth Medical School, 1992 Postdoctoral Fellow, Stanford University Yeast Actin cytoskeleton Assembly and Organization The actin cytoskeleton is the major organizing structure of the eukaryotic cytoplasm and its integrity is required for a number of cullular processess. Richard L. Cross, Ph.D. Yale University, 1970 Postdoctoral Fellow, UCLA Oxidative Phosphorylation/ Nucleotide-binding Sites/Enzyme Mechanisms A major goal of our research program is to derive a better understanding of the structure, regulation, and function of the membrane-bound protein complex that synthesizes ATP during oxidative phosphorylation and photophosphorylation. Other research interests in red cell physiology include membrane pathologies associated with congenital hemolytic anemias and systemic diseases, the cytotoxic effects of calcium, determinants of red cell life-time and survival, red cell shape, membrane lipid fluidity, the development of membrane transport properties, neonatal red cells, cell volume regulation, and reconstitution of red cell transport systems into planar lipid bilayers. Peter J. Hahn, Ph.D. University of California at Davis, 1981 X-Ray/Mutagenesis/Drug Resistance/Gene Amplification in Cultured Mammalian Cells Research in our laboratory focuses on the molecular mechanisms underlying the large-scale chromosomal rearrangements associated with oncogene over-expression in tumor cells, and drug-resistance in cultured mammalian cells. Patricia M. Kane, Ph.D. Cornell University, 1987 Organelle Acidification/ Vacuolar H+-ATPase/ Assembly and Transport of Multisubunit Proteins in Yeast Vacuolar ATPases acidify intracellular compartments such as the lysosome, endosome, Golgi apparatus, and clathrin-coated vesicles in all eukaryotic cells, and are also important in the specialized function of certain cells of bone, kidney, and brain. The assembly and transport of the yeast vacuolar H+-ATPase are particularly interesting because the enzyme is composed of residents of two different intracellular environments: cytoplasmically-oriented peripheral subunits, which do not appear to enter the secretory pathway, and integral membrane subunits that might be expected to act as vacuolar membrane proteins, traveling through the initial stages of the secretory pathway en route to the vacuole.

Smith has characterized mutations that result in the severe, neurological form of Gaucher disease. The success of such therapies will depend on the ability of the cells which contain the corrective gene to deliver the enzyme to their neighbors; Dr Smith is optimizing parameters for correctly expressing betaGlc in brain cells. Doll, R. and Smith, F.I. Regulation of expression of the gene encoding human acid beta-glucosidase in different cell types. Doll, R.F., Bruce, A. and Smith, F.I. Regulation of the human acid b-glucosidase promoter in multiple cell types. Lees, M.B., Smith, F.I., Dyer, C.A., Greer, J.M. and Pakaski, M. Expression of myelin proteolipid protein in oligodendrocytes and transfected cells. Doll, R.F., Crandall, J.E., Dyer, C.A., Aucoin, J.M.. and Smith, F.I. Comparison of promoter strengths on gene delivery into mammalian brain cells using AAV vectors.

J.B. Lloyd, C.L. Andrew, A.R. Klemm The lysosome membrane contributes to the efficiency of macromolecule degradation in lysosomes. The final products however are able to traverse the membrane and thus contribute significantly to metabolic pools in the cytoplasm. Over the past decade a number of metabolite porters in the lysosome membrane have been described. The information gained will also assist the development of drug-delivery systems in which drug conjugates are targeted to the lysosomes by receptor-mediated endocytosis: in such systems it is essential that the drug can leave the lysosome, and this is unlikely to occur except by passive diffuison. Physiological substances such as glycerol and the pentoses appeared to cross the membrane rapidly by passive diffusion, a conclusion concordant with the observation that no carriers have been identified for these metabolites in the lysosome membrane. In continuing work the ability of a range of organic molecules to cross the lysosome membrane is being studied.

The Office of Disease Prevention and Health Promotion, Office of Public Health and Science, Office of the Secretary, U.S. Department of Health and Human Services, works to strengthen the disease prevention and health promotion priorities of the Department within the collaborative framework of the HHS agencies. Announcements healthfinder™, a gateway web site to consumer health information from the United States government and many not-for-profit organizations, was launched April 15th by Secretary Donna E. Shalala at the 1997 Partnerships for Networked Consumer Health Information Conference. For the latest information on the Healthy People 2000 Consortium, visit the Healthy People 2000 home page. After 1 year of operation, this site has been incorporated in the new healthfinder™. Feedback: Please send comments/suggestions to David Baker.

In general all information presented in these pages and all items available for download are for public use. However, you may encounter some pages that require a login password and id. If this is the case you may assume that information presented and items available for download therein are for your authorized access only and not for redistribution by you unless you are otherwise informed. (404)639-3311 Send questions or comments on specific public health topics to CDC's individual Centers, Institute, and Offices.

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--> --> The Hereditary Disease Foundation is a non-profit, basic science organization dedicated to the cure of genetic disease. A centerpiece of the Foundation is the program of interdisciplinary Mary Jennifer Selznick workshops held many times during the year. These small, informal, free-wheeling workshops foster dialogue among researchers, from a variety of fields, who come without prepared lectures or slides to converse across disciplinary borders. Because Huntington's disease destroys so many different capabilities - intellectual, physical and emotional - the insights gained from research on this illness are relevant to the understanding of many others, including schizophrenia, manic depression, Alzheimer's, amyotrophic lateral sclerosis (Lou Gehrig's disease), Parkinson's disease, and cancer. Following that momentous discovery, the Hereditary Disease Foundation organized the Huntington's Disease Collaborative Research Group, which in 1993 captured the Huntington's disease gene - one of a newly identified class of disease genes whose signature is an excess of trinucleotide repeats, for example, the genes for Fragile X, the most common form of mental retardation, myotonic dystrophy, Kennedy's disease (spinobulbar muscular dystrophy), Haw River Syndrome, 3 types of hereditary ataxia, SCI1, Machado Joseph disease and Friedrich's ataxia. Through grants, fellowships, and the workshop program, the Foundation continues to build a research community committed to the cure of Huntington's disease and related disorders.

Research: This research addresses characteristics of lysosome-associated membrane proteins (LAMPs) expressed at the cell surface, their significance in autoimmune diseases and as markers for cell activation, and their role in cell adhesion and cytotoxicity. Kim is cloning and expressing wild type and mutant forms of the major regulatory protein of equine herpesvirus type 1 in order to ascertain which domains of this protein interact with DNA sequences upstream of other viral genes to regulate their expression. These studies will reveal how viral regulatory proteins function alone and in concert with cellular regulatory factors in herpesvirus infection. The goal of the project is to identify key viral proteins that elicit a protective cell mediated response and to use molecular approaches to generate viral mutants that will express these polypeptides and lack specific viral genes that are not essential for virus replication but play a role in viral pathogenesis. Wang's work is focused on identifying the extracellular signals responsible for driving T cell development and characterizing their precise role in this process. Wang will apply his work in murine T cell development toward establishing an in vitro model of early T cell development in human bone marrow.

History of Celiac Disease Glossary of Terms Celiac Disease: What it is, What Causes it, How it Affects People Diagnosis of Celiac Disease Treatment of Celiac Disease -- For Parents Treatment of Celiac Disease -- For Children Treatment of Celiac Disease -- For Teens Long-Term Problems Associated with Celiac Disease What's Gluten-Free and What's Not Support Groups/Recipes Books/Booklets Food Companies Celiac disease (Sprue): A disease in which certain proteins found in grains such as wheat, barley, rye, and oats, act as antigens. Damage to the intestinal mucosa interferes with digestion and absorption of food into the system, allowing undigested food to pass through the body into the stools (malabsorption). In people with celiac disease, gliadin and similar proteins in rye, oats, and barley work as antigens. Diagnosis of celiac disease requires a small intestinal biopsy, which demonstrates the typical pattern of intestinal mucosal injury (villus atrophy), and a clear-cut disappearance of symptoms and restoration of growth and weight gain occurring over a matter of weeks to months on a gluten-free diet. Thereafter, gluten may be temporarily reintroduced and a third biopsy performed weeks to months later in order to see if re-exposure of the intestine to gluten results in a return of the villus atrophy.

Lysosomes are protein-containing sacs produced by the activity of the rough endoplasmatic reticulum (rough ER) and the Golgi complex. In these two compartments, proteins, synthesized in the ribosomes, are distributed or further chemically modified by the attachment of groups, such as sulphates, and lipid or sugar units. Remarkably, mitochondria contain their own DNA (mtDNA) encoding for a small but vital set of proteins. Several discrete mutations in mtDNA and nuclear DNA have been described which affect the protein household of mitochondria. After it was established that the rare Zellweger's cerebrohepatorenal syndrome is associated with the absence (or at least dysfunction) of peroxisomes, the complex function of peroxisomes in dealing with the toxic substance hydrogen peroxide and with etherphospholipid synthesis, fatty acid oxidation and glyoxylate metabolism, was finally correlated with congenital diseases. Forward to: Ubiquitin, stress proteins and the cytoskeleton Back to: The growth of cytology Return to: Cell biology in clinical medicine contents page

The intraluminal region is formed by two homologous domains separated by a proline-rich hinge region. There are 18 potential N-linked glycosylation sites, some of which link poly-N-acetyllactosamines. PROTEINS AND DNA ELEMENTS WHICH REGULATE TRANSCRIPTION OF CD107b MOLECULECOMMENT SP1 Potential SP1 and AP-1 binding sites present in the 5'-flanking region (-172 to -20 bp). AP-1 Potential SP1 and AP-1 binding sites present in the 5'-flanking region (-172 to -20 bp). KpnI A KpnI repeat sequence upstream of the promoter sequence might regulate expression (Sawada et al. DISEASE RELEVANCE OF CD107b AND FUNCTION OF CD107b IN INTACT ANIMAL Highly metastatic tumor cells express more lamp molecules on the cell surface than poorly metastatic cells.

Rush Alzheimer's Disease Center: Alzheimer's related web sites

Karl Johnson, Virologist, Epidemiologist, Centers for Disease Control (retired)Tim Cochnauer, Regional Fishery Manager, Clearwater Region, Idaho Department of Fish and Game Description: Rocky Mountain Wreck: Exotic Parasite has Wild Trout in a Whirl Myxobolus cerebralis arrived in North America from Europe 40 years ago. It has slowly spread across 21 of our northern states, largely from trout hatcheries. The ubiquitous tiny worm, Tubifex, serves as crucial alternate host for this durable parasite, which has finally invaded many of the best rivers in the region where fisheries are managed as wild, naturally reproducing populations. The present and predicted extent of damage and the prospects for restoration of viable wild salmonids will be surveyed.

Heretogeneous calcium flux in peripheral T cell subsets as revealed by 5 color flow cytometry using log ratio circuitry. Use of an individual viable cell fluorogenic assay (FACS-Gal) to study HIV gene regulation and assay for infections virions. 10 (supplement 1), p. S23. 10 (supplement 1), p. S27. 10 (supplement 3), p. S109. 10 (supplement 3), p. S109.

Gross Pathology: Necropsy examination performed on one of the calves by the attending veterinarian revealed petechial hemorrhages on the serosal surfaces of the spleen, intestines, omentum and the medulla of the kidneys. Fresh and formalin fixed tissues including lung, liver, kidney, spleen and small intestine were submitted. AFIP Diagnosis: Kidney, medulla: Vasculitis, necrotizing, acute, diffuse, moderate, with hemorrhage, fibrin thrombi, subacute tubulointerstitial nephritis, tubular proteinosis, and endothelial basophilic intranuclear inclusion bodies, Holstein, bovine, etiology consistent with adenovirus. After transmission, infection, and a viremic stage, adenovirus localizes in the endothelial cells of many organs (including the intestines, stomach and forestomachs, adrenal glands, lymph nodes, liver, spleen, urinary bladder, and kidneys) resulting in thrombosis and ischemia. Laboratory Results: Nocardia asteroides was cultured from the subcutaneous lesions, lymph nodes, kidney, liver, lung and spleen. Pyogranulomatous inflammation with branching, filamentous, gram-positive bacteria was also identified microscopically in the lung, kidney, lymph node and skin lesions.

We study transcription factor interactions in the control of gene expression in human cells. Studies are focused on the molecular genetics and function of the two arginase genes in man and other mammals, and use the techniques of cell culture, recombinant DNA, and gene transfer. Current efforts are being directed towards identifying essential biochemical properties of EWS/FLI on growth and differentiation of neural crest cells and isolating potential target genes that are modulated by EWS/FLI. Nature Genetics 6:146-151, 1004 Lessnick SL, Braun BS, Denny CT and May WA: Multiple domains mediate transformation by the Ewing's sarcoma EWS/FLI fusion gene. The gene plays a major role in cell cycle progression, cancer susceptibility, Purkinje cell development, DNA repair, sensitivity to ionizing radiation, and thymic maturation. REPRESENTATIVE PUBLICATIONS: Varnum B.C., Ma Q, Chi T, Fletcher B, Herschman H.R.: The TIS11 primary response gene is a member of a gene family that encodes proteins with a highly conserved sequence containing an unusual cys-his repeat.

We study transcription factor interactions in the control of gene expression in human cells. Studies are focused on the molecular genetics and function of the two arginase genes in man and other mammals, and use the techniques of cell culture, recombinant DNA, and gene transfer. Current efforts are being directed towards identifying essential biochemical properties of EWS/FLI on growth and differentiation of neural crest cells and isolating potential target genes that are modulated by EWS/FLI. The gene plays a major role in cell cycle progression, cancer susceptibility, Purkinje cell development, DNA repair, sensitivity to ionizing radiation, and thymic maturation. REPRESENTATIVE PUBLICATIONS: Varnum B.C., Ma Q, Chi T, Fletcher B, Herschman H.R.: The TIS11 primary response gene is a member of a gene family that encodes proteins with a highly conserved sequence containing an unusual cys-his repeat. Our laboratory is involved in the molecular analysis of human diseases affecting the nervous system using physical mapping and linkage techniques, as well as biochemical and anatomical characterization of the gene products.

Marvin Natowicz was on call when a week-old baby girl was rushed into the newborn intensive care unit. I'm a pathologist," says Natowicz, who is currently assistant professor of pathology at Harvard Medical School and director of medical genetics at the Eunice Kennedy Shriver Center. Thinking excess waste could be due to a lack of one of the lysosomal enzymes that typically digest cellular waste, Natowicz set off in search on a search for the missing enzyme. Reasoning that the girl's masses-which were located around the joints-could be due to a lack of hyaluronidase, he and his colleagues spent a year developing an accurate hyaluronidase test. In the companion study, Natowicz and his colleagues found that people who-owing to a rare set of genetic disorders-lack the ability to put mannose-6-pathway recognition tags on their enzymes have very high levels of most lysosomal enzymes in their blood. "Hyaluronidase must be getting to the lysosome by some other pathway," says Natowicz.

Thursday, September 19, 1996 Reception/buffet Overview session Friday, September 20, 1996 Endoplasmic reticulum Cell Polarity Poster session Exocytosis Saturday, September 21, 1996 Golgi and TGN Endocytosis and vacuole/lysosome Thursday, September 19, 1996 4:30-6:15 pm Reception/buffet in Severance Hall 6:30-9:15 pm Session I: Overview session (Allen Memorial Library) T. Rapoport (Boston) Transport of proteins across the endoplasmic reticulum membrane D. Williams (Toronto) Molecular chaperons of the ER in health and disease Coffee breakK. Sandvig (Oslo) Endocytosis and intracellular transport of protein toxins J. Fox (Cleveland) Cytoskeleton and membrane insertion A. Tartakoff (Cleveland) Lipid distribution along the secretory path G. Warren (London) Mitotic disassembly and reassembly of the Golgi apparatus V. Malhotra (San Diego) Biogenesis of Golgi stacks by novel vesiculation and fusion events Coffee break J. Bonifacino (Bethesda) Recognition of sorting signals by membrane coat proteins P. Cresswell (New Haven) Mechanism of MHC class-II restricted antigen processing W. Macklin (Cleveland) Sorting of myelin proteins in oligodendrocytes P. Davis (Cleveland) Utilizing the cellular machinery for therapeutic purposes: receptor-mediated gene transfer S. Younkin (Jacksonville) The role of altered amyloid precursor protein processing in Alzheimer's disease W. Wickner (Hanover) Mechanism of vacuole inheritance D. Russell (St Louis) Phagosome biogenesis and its perversion by Mycobacterium and Leishmania This page was last updated September 3, 1996.

The symptoms of Tay-Sachs Disease Life's molecules Normal cell function Cell function changes in Tay-Sachs How does studying diseases help us to understand how cells function normally? Tay Sachs Disease I mentioned in the first lecture about genetic screening to identify carriers of Tay Sachs. Let's consider Tay-Sachs again and talk about how cells function. People with TS have abnormal brain cells and retinas OH of red spots (on fovea centralis) and bodies in cells bodies in cells are gangliosides which involved in nervous system allowing cells to recognize each other normally this lipid-sugar is broken down by an enzyme, hexosaminidase but for some reason it accumulates in nervous system cells To understand we need to follow the pathway of this special lipid sugar which involves a series of organelles (membrane-bound structures in cells).

How HIV Causes AIDS An important focus of the National Institute of Allergy and Infectious Diseases (NIAID) is research devoted to the pathogenesis of human immunodeficiency virus (HIV) disease -- the complex mechanisms that result in the destruction of the immune system of an HIV-infected person. A detailed understanding of HIV and how it establishes infection and causes the acquired immunodeficiency syndrome (AIDS) is crucial to identifying and developing effective drugs and vaccines to fight HIV and AIDS. This fact sheet summarizes what scientists are learning about this process and provides a brief glossary of terms. Overview HIV disease is characterized by a gradual deterioration of immune function. Most notably, crucial immune cells called CD4+ T cells are disabled and killed during the typical course of infection. These cells, sometimes called "T-helper cells," play a central role in the immune response, signalling other cells in the immune system to perform their special functions.

New cells come from the division of old cells. Cells with a nuclear envelope are called eukaryotic cells. Cells without a nuclear envelope are called prokaryotic cells The cytoplasm of eukaryotic cells is also more complex than that of prokaryotic cells. Eukaryotic cells are also much larger than prokaryotic cells. NUCLEUS is the site of DNA, genetic material RIBOSOMES -- site of protein synthesis ROUGH ENDOPLASMIC RETICULUM-- membrane sacs that make proteins found in proteins or exported from the cell SMOOTH ENDOPLASMIC RETICULUM-- makes lipids and cell membranes GOLGI APPARATUS -- modifies newly made proteins, lipids, and carbohydrates VESICLES -- membrane balloons that transport substances in cells LYSOSOMES -- sacs contaiing enzymes that digest worn out cell parts CYTOSKELETON -- fibers made of protein that help cell maintain its shape MITOCHONDRIA -- organelles that harvest energy from organic molecules

Note the row of red blood cells in the capillary in the lower portion of the field. TEM of connective tissue cells (Erlandsen and Magney, Human Histology: A Microfiche Atlas). Note the distinct columnar shape of the cells in this epithelium and the relatively uniform location of the nuclei. Note that this columnar epithelium (unlike that of the gallbladder) contains mucus-secreting goblet cells (see Micro 37) in addition to absorptive cells that possess numerous apical microvilli (see Micros 38-40). Note the difference in the amount of space between the epithelial cells versus the space between the connective tissue cells. Note the variety of different connective tissue cells in this field.

A 17 year old female, adopted at age 21 months, who had normal development until age 8 years when her parents initially noted the onset of difficulties with balance and hearing. Since then, she has experienced a progressive neurologic deterioration manifesting as dysarthria, dysphagia, ataxia, supranuclear vertical gaze paralysis and a global cognitive deficit. She has been wheelchair bound since age 15 years. Her workup at an outside institution included: an unremarkable MRI scan of her brain a normal muscle biopsy a normal urine metabolic screen and a normal karyotype Upon referral to the Neurology Clinic at the University of Michigan Medical Center, she was noted to have: ataxia of speech, extremities and gait; vertical gaze supranuclear palsy; global cognitive deficits; and splenomegaly. The differential diagnosis of foamy histiocytes in the bone marrow includes: Niemann - Pick disease Other storage diseases (e.g. Hurler's disease) Hypercholesterolemia Hyperlipoproteinemia Lipogranulomas Sea blue histiocyte in bone marrow aspirate (20x magnification). The differential diagnosis of sea blue histiocytes in the bone marrow includes: Niemann - Pick disease Other storage diseases (e.g. Hurler's disease) Myelodysplasia Chronic myelogenous leukemia Hyperlipoproteinemia Hypercholesterolemia

We remember Rudolf Virchow LEAST for: A. being the founder of modern-day pathology B. his idea that all cells come from pre-existing cells C. his idea that all disease is disease of cells D. his idea that politics is a major cause of human disease * A. WBC 2000, 90% segmented neutrophils, no bands, the rest lymphocytes and monocytes B. WBC 5100, 52% segmented neutrophils, many with six or more lobes to their nuclei * C. WBC 9000, 52% segmented neutrophils, 32% bands D. WBC 11,000, 52% segmented neutrophils, 17% lymphocytes, 1% eosinophils, 1% basophils, 29% macrophages E. WBC 15,000, 98% segmented neutrophils, 1% lymphocytes, 1% monocytes 54. Hereditary lack of protein C is a notorious cause of A. inability to clot the blood B. inability to fix complement C. inability of neutrophils to reach the invaders D. inability to kill many viruses * This lesion from lung is probably a(n): A. acute inflammation (pneumonia) B. cancer, you can tell by the anaplasia C. hemorrhage without infarction, as in Goodpasture's disease * This kidney lesion is a(n): A. abscess B. calcification, probably dystrophic C. calcification, probably metastatic D. granuloma *

a-L-Iduronidase participates in the stepwise degradation of the glycosaminoglycans, dermatan sulfate and heparan sulfate, within lysosomes; its absence interrupts the degradative pathway and causes lysosomal storage of undegraded substrate, with resulting pathology of cells, tissues and organs. A milder form of a-L-iduronidase deficiency, the Hurler/Scheie syndrome, is compatible w ith normal intelligence and survival to adulthood albeit with severe physical handicaps, while the Scheie syndrome is milder yet and may permit a normal life. There are two widely distributed mannose 6-phosphate receptors, which target endogenous as well as endocytosed enzyme to lysosomes (18,19); other carbohydrate receptors, which are specific for endocytosis, include the galactose (asialoglycoprotein) rece ptor of hepatocytes (20) and the mannose receptor of macrophages (21). There are two widely distributed mannose 6-phosphate receptors, which target endogenous as well as endocytosed enzyme to lysosomes (18,19); other carbohydrate receptors, which are specific for endocytosis, include the galactose (asialoglycoprotein) rece ptor of hepatocytes (20) and the mannose receptor of macrophages (21). There are two widely distributed mannose 6-phosphate receptors, which target endogenous as well as endocytosed enzyme to lysosomes (18,19); other carbohydrate receptors, which are specific for endocytosis, include the galactose (asialoglycoprotein) rece ptor of hepatocytes (20) and the mannose receptor of macrophages (21). There are two widely distributed mannose 6-phosphate receptors, which target endogenous as well as endocytosed enzyme to lysosomes (18,19); other carbohydrate receptors, which are specific for endocytosis, include the galactose (asialoglycoprotein) rece ptor of hepatocytes (20) and the mannose receptor of macrophages (21).

QUESTION #5 (6 min) Green fluorescent protein (GFP) from the marine invertebrate Aquoria victoria has become a popular "reporter" protein to study protein targeting in eukaryotic cells. a head group moves from one side of the membrane to the other side 1 point e) a lipid bilayer consists of two leaflets, with the hydrophobic tails facing each other and forming the hydrophobic interior The composition of the two lipid leaflets is usually different with respect to individual types of lipid molecules 2 points f) 7 - 10 nm ( imagine two carbon chains, of 16 -20 carbons, arranged end to end: at least 32 - 40 C-C bonds of ~1.5 Angstroms, ie a total of >30 - 100 Angstroms, or 10 nm) 1 point QUESTION #2 a) a protein made on free polysomes is not integrated into the membrane to be associated with the membrane, it has to become attached to an integral membrane protein ==> peripheral membrane protein OR, it can be modified by a covalent attachment (amide bond) of either a fatty acid (side chain myristyl- ) at the N-terminal, or a farnesyl group via a thioether linkage to a cysteine at the C terminal 4 points b) a peripheral membrane protein can be solubilized at high salt or extreme conditions of pH a protein anchored by a myristyl or farnesyl chain must be solubilized by a detergent 2 points c) both of these proteins would be associated with the interior of the plasma membrane, and hence untouched by trypsin applied on the outside 4 points QUESTION #3 the nucleotide sequence from the gene or from a cDNA would allow us to deduce the complete amino acid sequence for the protein. an integral membrane protein might be expected to show two features: i) a sequence of ~ 20 hydrophobic amino acids at the N-terminal serving as a signal sequence for binding to the SRP, and to be inserted into the RER membrane ii) in addition, we need to have at least one internal segment of the polypeptide containing a stretch of ~ 20 amino acids with hydrophobic side chains; these form the alpha- helical transmembrane segment of the IMP; if more than one such stretch of aa is found, the integral membrane protein may have multiple transmembrane segments 6 points QUESTION #4 a) facilitated diffusion requires a permease or carrier protein characteristics of an enzyme: substrate specificity, exhibits saturation kinetics, characterized by parameters such as Vmax and Km 3 points b) chloride/bicarbonate exchange is an example of antiport 2 points c) ATP hydrolysis occurs on one side of the membrane protons are pumped from low to high concetration the pH is higher on the side where ATP is hydrolysed 4 points d) ligand-gated channels are opened by the binding of a ligand (small molecule, eg neurotransmitter) 2 points voltage-gated channels are opened by a change in membrane potential, ie the voltage across the membrane 2 points the flux of ions is determined simply by the gradient of ions across the channel, from high to low concentration 2 points the equilibrium is not simply between two states (open and closed), but there is a path of sequential conformational changes involving three states the refractile state is a closed state from the the channel cannot be opened directly 4 points QUESTION #5 a) In normal cells this GFP with the SKL sequence will be targeted to the peroxisome, and staining will be punctate, ie reflect the concentration of this protein in vesicles in cells from Zellweger patients the uptake of this normal protein into peroxisomes does not occur, because they lack an essential component for transport of these proteins into the peroxisome. 6 points QUESTION #6 the corresponding mRNAs are made in vitro and then microinjected into frog oocytes after some time to allow these mRNAs to be translated and to get these proteins to their final destination (the plasma membrane in this case) we can look for new properties of these oocytes which are not observed with normal oocytes or with oocytes injected with pure water if a water channel is expressed, the oocytes will swell quickly to the point of bursting when placed into hypotonic solution (in this case no radioactive glycerol will be taken up into the eggs) if a glycerol channel is expressed, we can measure the uptake of radioactive glycerol into the oocytes ( in this case exposure to hypotonic solution will not burst the oocyte) the expression of the desired proteins was ascertained after labeling the oocytes with a radioactive amino acid, and detecting novel proteins of the right size by gel electrophoresis of total cell extracts. ) 12 points QUESTION #10 a) one can translate a mitochondrial protein in vitro in the absence of any mitochondria (making it radioactive in the process by the inclusion of a radioactive amino acid in the translation mix); this protein can be digested when exposed to trypsin If isolated, energized mitochondria (from yeast cells, for example) are added AFTER protein synthesis is complete, the protein will be taken up into the mitochondria. Other organelles importing proteins that are complete are: peroxisomes, nuclei 4 points b) we require: hsp70 protein (chaperone) in the cytosol to denature the protein to be imported and remove secondary and tertiary structure the mitochondrial targeting sequence on the protein has to be recognized by a receptor on the outer membrane a membrane complex has to be assembled to make an aqueous channel through the outer and inner membrane (at the points of close contact between these two membranes) on the inside, the unfolded peptide is first bound again by a mitochondrial hsp70 protein, but later released to the hsp60 (chaperonin complex) which refolds the peptide into a biologically active secondary and tertiary structure 4 points QUESTION #11 a) nuclei import proteins made in the cytosol and export polynucleotides (mRNA and tRNA) into the cytosol.

(April 23) Lysosome (not a microbody)   Discovery, Christian de Duve, 1945-50 Acid phosphatase "latent" enzyme activity Structure single membrane average 0.5 micron electron dense Origin trans Golgi cisternae mannose-6-phosphate mannose-6-phosphate receptor I-cell disease Primary lysosome Secondary lysosome Relation to endocytosis Functions: Phagocytosis phagocytotic vacuoles Autophagy autophagic vacuole Autolysis programmed cell death Extra-cellular digestion rheumatoid arthritis hydrocortisone Silicosis - minors disease asbestos Lysosomal storage diseases: Type II glycogenosis alpha-glucosidase Hunter's Syndrome alpha-fucosidase Hurler's Syndrome alpha-L-iduronidase Tay-Sach's disease B-N-acetylhexosaminidase A      

Beaman retains any copyright to these images, and presentation here does not imply a right to copy them. Here is a picture of a colony of this organism growing on an agar plate. Nocardia is an Actinomycete - a bacterium that grows filamentously, rather like the eukaryotic fungi, only smaller. This is a SEM picture of Nocardia getting phagocytosed by an immune system cell. You can see around these cells a light gray area where there are no organelles - especially the numerous dark circular organelles - the lysosomes (labelled L). In these cells, the lysosomes do fuse with the phagosomes, and result in the destruction of the bacteria.

Introduction Mucopolysaccharide Storage Diseases Symptoms Incidence How MPS Diseases are Inherited Genetic Counselling and Prenatal Diagnosis Mucolipidoses The Society for Mucopolysaccharide Diseases, Inc. Because of the isolation I felt when my second child was diagnosed with MPS, I founded the national Society for Mucopolysaccharide Diseases, Inc. The Society has allowed me to have contact with other MPS families. It can acquaint carrier couples with other options of having children: prenatal diagnosis by amniocentesis and selective abortion, adopting, artificial insemination of taking the 25% chance in each pregnancy of giving birth to an MPS child. 1968 Co-culturing of fibroblasts from Hunters patients with fibroblasts from Hurlers patients corrected this accumulation of GAGs, i.e. the enzyme defect in Hunters patients could be overcome by an enzyme present in Hurler patients, and vice versa. Multiple Sulphatase Deficiency shown to be defective for many enzymes including idurono-sulphate sulphatase (this enzyme is defective in Hunters syndrome), Arylsulphate A (this is defective in Metachromatic Leukodystrophy), Arylsulphatase B (this is defective in Maroteaux-Lamy), Arylsulphatase C, and Cholesterol sulphatase.

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The Department of Gynecologic Oncology provides state-of-the-art treatment to women with gynecologic cancer, directs the Gilda Radner Familial Ovarian Cancer Registry, conducts clinical research in an attempt to afford women the highest possible chance for cure, while at the same time doing basic science research in an attempt to discover the etiology of gynecologic malignancies. Using information from families from the Gilda Radner Familial Ovarian Cancer Registry, and in collaboration with researchers at the University of Cambridge, we are attempting to further localize the breast/ovarian cancer susceptibility gene (BRCA-1) on 17q12-21 in families with familial ovarian cancer. Using intraperitoneal cisplatin and cytarbine in women with recurrent or persistent ovarian cancer after initial chemotherapy, we reported a five-year survival rate of 60% in patients who responded to first-line chemotherapy and then to second-line chemotherapy. In our report from 1981-1991, of the first 658 families, including 1,568 cases of familial ovarian cancer, we reported that the mean (58.5 years) and the median (57.0 years) ages at diagnosis of ovarian cancer of the mothers was significantly older than the mean (49.8 years) and the median (49.0 years) ages of their daughters with ovarian cancer, suggesting that surveillance by careful pelvic examination, CA125 blood test and ultrasound of the ovaries in the daughters of mothers with ovarian cancer needs to be started by age 25. Because women with two or more first-degree (mother, sister, daughter) or first- and second-degree (grandmother, aunt) relatives have a lifetime risk for developing ovarian cancer which approaches 50%, the Registry continues to recommend that such women undergo prophylactic oophorectomy by age 35, if they have completed their family. One research interest is to determine the molecular requirements for routing alpha-L-fucosidase from the start of synthesis in the rough endoplasmic reticulum to ultimate residence in lysosomes or extracellular fluids and to determine precise biochemical defects in routing of alpha-L-fucosidase in disease.

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An important constituent of the neuritic plaques is -amyloid, or A (1), a set of oligopeptides of about 40 to 43 amino acids that are proteolytically derived from a much larger -amyloid precursor protein (APP). The APP protein on the surfaces of appropriate neurons and the S182 (or alternatively the STM2) proteins on the surfaces of neighboring auxiliary cells [perhaps the microglial cells discussed by Cras et al. Instead, as a by-product of the intercellular interaction of APP and S182 (or STM2), perhaps by the process of mutual capping (13) of the two proteins into the membrane regions of cell-cell contact, vesicles would be pinched off the cell surfaces and incorporated into the interior of the neuronal cell (see figure). These vesicles would then fuse with multivesicular bodies inside the neuronal cell, where the APP would then be proteolyzed by enzymes in the multivesicular bodies, A being a product of this proteolysis. Furthermore, because APP is a ubiquitous cell surface protein of neurons, it is not clear how the selective deposition of the plaques in specific regions of the brain would occur. It can explain selective production of A in the hippocampus and adjoining cortex if the specific interaction between APP and S182 or STM2 required a particular form of regionally expressed APP [perhaps APP 695 (16)], S182, or STM2. The production of A as a product of the cell-cell interaction system would be distinct from A production by the normal turnover of APP on neurons and might therefore occur at a much slower rate, consonant with the usual late onset of AD.

News Search  Trades as of:May 02, 1997 As of: May 05, 1997 06:32 AM DJIA 7071.20 NYSE 422.97 NASDAQ 1305.33 Russell 2000 353.98 S&P 500 812.97 AMEX MMI 740.68 Announces Third Quarter Results and Write-Down of China Development Program BW   05/02 19:54 Cypress Bioscience announces increased sales, reduced costs and management investment in company stock PRN  05/02 19:34 E-Z Serve Reports Improved First Quarter 1997 Operating Results BW   05/02 19:16 SI Diamond announces first quarter results more headlines News powered by News Alert Inc. Delayed quotes powered by PC Quote Inc. All stock quotes are 20 minute delayed and are believed accurate but are not warranted or guaranteed by News Alert Inc. Lycos is a trademark of Carnegie Mellon University

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H.R.Taylor, "Studies on the tear film in climatic droplet keratopathy and pterygium," Arch. H.R.Taylor, S.K.West, F.S.Rosenthal, B.Munoz, H.S.Newland and E.A.Emmett, "Corneal changes association with chronic UV irradiation," vArch Opthalmol. O.L.Jenson, "Pterigium, the donimant eye and the habit of closing one eye in sunlight," Acta Opthalmol. P.Austin, F.A.Jacobiec and F.Daxecker, "Elastodyplasia and elastodystrophy as the pathologic bases of ocular pterygia and pinguecula," Opthalmol. Y.Guex-Crosier and C.P.Herbort, "Prsumed corneal intraepithelial neoplasin associated with contact lens wear and intense ultraviolet light exposure," Br. D.Sliney, "Physical factors in cataractogenesis: Ambient ultraviolet radiation and temperature," Invest.

While formulas are an excellent alternative when breast feeding is not possible, human breast milk is the superior food for human babies for many, many reasons. Even though many children have periods when they are quite picky eaters, most children get the minimum known requirements for vitamins from the foods they eat. Infant formulas already contain vitamin supplements, and while some believe that breast milk fails to provide adequate amounts of vitamins A, C, and D for young infants, I believe that breast milk is an ideal human food, and that our knowledge of nutritional requirements is lagging. Your son's weight puts him at the 10th percentile of healthy American children (Out of 100 healthy children his age, he would weigh less than 90 of them, but more than 10). They are used to increase appetite primarily in HIV-positive children or in children with cancer. Occasionally, human infants and children become infected with the dog tapeworm.

BS 11 Review Topics for 2nd Midterm Self-assembly symmetry, axis of symmetry structures that form via SA reasons to use SA Actin +, - ends critical concentrations Plant vs animal cells Muscle names, dimensions for various parts proteins actin myosin S1 fragment decoration of actin tropomyosin troponin complex - I, T, C components other stuff - i.e. titin, nebulin mechanism contraction cycle T-tubules sarcoplasmic reticulum tension vs length graph creatine phosphate - role in storing energy chickens Actin structures microvilli role in lamellapodia-based cell movement labelling experiments Microtubules filaments, protofilaments alpha, beta monomers centrosomes, basal bodies cillia/axoneme structure dynamic instability motor proteins dynein: - end directed motor kinesin: + end directed motor Karyokinesis centromere/kinetochore spindle formation role of motor proteins Phospholipids structure - hydrophillic head, phosphate, glycerol, 2 fatty acid tails repeating structures - micelles, tubes, monolayers, bilayers movement of lipids in bilayer earwax exper. - measures area of 1 lipid mol root hair experiment (Overton) blood cell experiment (Gorter/Grendel) fish egg exper. - measures membrane strength Transmembrane proteins Band III network in red blood cell EM studies of membranes labelling experiments scalar vs vectorial reactions Intercompartmental transport gated - i.e. to nucleus transmembrane - i.e. to ER vessicular - i.e. between ER and Golgi Cotranslational insertion (of protein into ER) SRP SR polysomes gel experiments under various conditions transmembrane proteins glycosylation of protein Protein sorting in Golgi default processing: constitutive secretion KDEL signal (go back to ER) Other sorting directions lysosome I cell disease secretory vessicles Parts of Golgi, events in var. compartments cis - remove mannoses medial - add GlcNAc

K. Sol-Church, G. Bertenshaw (to July 1996), J. Shipley The lysosomal proteinases are normally controlled by packaging into the lysosome, a discrete compartment within the cell. In a number of degenerative diseases such as rheumatoid arthritis, muscular dystrophy and a variety of cancers, enzymes escape the normal packaging mechanism and their uncontrolled activity results in excessive tissue damage. Fortunately, aberrant degradation by secreted forms of these enzymes is usually controlled either by naturally occurring inhibitors or by the secreted form being an inactive precursor. Our efforts have concentrated upon a project to explore the role of the N-terminal portion of cystatin C, the primary natural inhibitor of lysosomal proteinases, in this inhibition. Results from this study show that we can generate more specific inhibitors of cathepsins L and S by altering the amino acids that can bind in the specificity pockets of the enzymes. In a second project, we determined that the activation of pro-cathepsin L is controlled by one or more of six histidine residues in the N-terminal portion of the protein.

Suspected IEM with Metabolic Acidosis, Diagnostic Flowchart The presence of metabolic acidosis is an important finding and the starting point for one of the two algorithms. Galactosemia and Lowe's syndrome (oculocerebrorenal syndrome, an X-linked recessive disorder with congenital cataracts, proximal RTA, and mental retardation) as well as many other metabolic disorders which present later are associated with a RTA, usually a proximal RTA. If these are normal (except for the changes found with lactic acidosis), then the lactate/pyruvate ratio and the glucose level will allow differentiation into 1) glycogen storage disease, gluconeogenesis disorders, or endocrine causes; 2) disorders of pyruvate metabolism; or 3) defects in mitochondrial energy metabolism. Suspected IEM with Neurologic Abnormalities and Without Metabolic Acidosis, Diagnostic Flowchart The neurologic abnormalities in the absence of metabolic acidosis is the starting point for the second major algorithm. Increased ammonia in the absence of acidosis is either due to a urea cycle defect or transient hyperammonemia of the newborn (THAN, a disorder of unknown etiology, more frequent in prematures, associated with profound hyperammonemia and coma). Fatty acid oxidation disorders, glutaric acidemia type II - patients may have dysmorphic features similar to Zellweger's, hypertrophic cardiomyopathy, and multicystic dysplastic kidneys.

Fungi: Answer D Are plants Usually contain chlorophyll Are often autotrophic Are often saprobes 2. Viruses belong to the kingdom: Answer D Protista Plantae Monera None of the above 3. All of the following are characteristics of at least some of the Monera EXCEPT Answer D photosynthesis. The outermost boundary of an animal cell is the Answer A plasma membrane nucleus cytoskeleton cell wall 12 What is the role of the transport and channel proteins within the fluid mosaic of a plasma membrane Answer C They prevent passage of amino acids. Answer C Prokaryotes have RNA, eukaryotes have DNA Prokaryotes have a nucleus, eukaryotes have a nucleoid Prokaryotes have a nucleoid, eukaryotes have a nucleus Prokaryotes have a cell wall, eukaryotes do not 35. When a virus takes over the machinery of a cell, it forces the cell to manufacture Answer D more mitochondria for energy for the virus.

(Ch 20) & p 112-115 Topics: ER Golgi signals - ER protein distribution signals - nucleus, mito exocytosis endocytosis Vesicles lysosomes membrane recycling Glycosylation Intro - Control of local environments (nucleus, mitochondria, cytoplasm, membrane, aqueous, outside . ) - sorting, distribution, modification processes Endoplasmic Reticulum (ER) - network of vesicles - continuous membrane system - tubules, vesicles, cisternae - > 1/2 total membrane - catalytic surface for biochemical activity - lumen separate from cytoplasm - 2 types; 1) rough - ribosomes - cisternae 2) smooth - sm. ) hydrophillic coating for aqueous travel Metabolic Sites; enzymes for - oxidation (fats) - glycolysis - steroid synthesis rER ==> protein synthesis sER ==> lipid synthesis, non protein synthesis - central, polar (concave/convex; cis/trans), flattened cisternae - avg 20/cell, microtubules hold place and shape move by dynein/kinesin - no ribosomes on or around - dynamic budding/fusion - rER sm protein filled transional vesicles > cis face - vesicles - transport proteins and lipids golgi - language (shuttle vesicles) - inverted in exocytosis - processing --> trans face --->secretory vesicles (lysosome, storage) - endocytosis --> prot. memb thin (ER) thick (plasma memb) pH neutral acid Fatty acylase + Mannosidase I + NADPase + Phosphatase + Adenylate cyclase + + acid phosphatase + Galactosyl transferase + Nucleoside diphosphatase + microbody -direct to ER, mito, chloro - N-ter deleted upon entry signature - combination of polar, nonpolar, charged aa, not aa specific Targetting the ER Signal Hypothesis Observations 1) new protein =/= secreted protein in size. Postinsertion Signals - difficult to work with KDEL (Lys-Asp-Glu-Leu) at C-term targets to ER and KDEL receptor - fig 20-11 - salvage Mannose 6-phosphate - added to protein destined for lysosome - golgi trans receptor binds (pH7) releases pH cells do not degrade in lysosomes - two examples of ER memb signals and results Mito & chloroplasts - protein synthsized in cytoplasm (not ER) - Mito signal fig20-13 - 2 receptors on outer memb (MOM19 & 72) - note if 2nd memb is bacterial --> distinct signals?

Answer A aerobic respiration anaerobic respiration alcoholic fermentation lactate fermentation 2. A property that all living organisms have in common is: Answer C They breath oxyegn They make glucose They contain DNA All of the above 3. Blue green Algae belong to the kingdom Answer A Monera Protista Fungi Plantae 4. Answer C Prokaryotes have RNA, eukaryotes have DNA Prokaryotes have a nucleus, eukaryotes have a nucleoid Prokaryotes have a nucleoid, eukaryotes have a nucleus Prokaryotes have a cell wall, eukaryotes do not   5. The outermost boundary of an animal cell is the Answer A plasma membrane nucleus cytoskeleton cell wall 40. When a virus takes over the machinery of a cell, it forces the cell to manufacture Answer D more mitochondria for energy for the virus.

Etiology Table 154.1 lists most of the clinically important drugs and chemicals known to produce nephrotoxicity. A partial list of such agents is presented in Table 154.2. Many agents are nephrotoxic via methemoglobin formation; a list is in Table 154.3. Allergy is involved in the acute tubulointerstitial nephritis (TIN) of the penicillins (especially methicillin), rifampin, sulfonamides, or combinations of trimethoprim and sulfamethoxazole (see Table 154.6). Mercury, bismuth, and thallium nephrotoxicity seem to be decreasing, but cadmium, copper, gold, uranium, arsenic, and iron nephrotoxicity are still prevalent --the last associated with proximal myopathy in hemochromatosis and in other forms of iron overload, eg, in dialysis patients with multiple transfusions. Drugs inducing immune complex disease in the kidney, proteinuria, and many features of nephrotic syndrome include penicillamine, captopril, levamisole, and gold salts used by injection for RA.

Examine structure of amyloid deposits and of the proteins that make them. NMR structure of the mouse prion protein domain PrP(121-231). In recent years, substantial evidence has shown that an essential component of the infectious agent is an alternate conformer (or possibly small aggregate) of PrP. PrP(C) is the normal cellular isoform of the prion protein, PrP(Sc). An important proof of the protein-only hypothesis was the demonstration that the incubation time for transmission from hamster to mouse was significantly shortened for a transgenic mouse bearing either hamster PrP gene or a hybrid mouse/hamster PrP gene. Mice are not infected by human prions, nor are transgenic mice bearing a copy of human PrP; however, transgenic mice bearing a hybrid mouse/human PrP are infected by human prions. This suggests that an interaction between a host factor and PrP is necessary for transmission and that the mouse factor is not sufficiently similar to the human factor to interact with the human PrP. Including some mouse sequences in the otherwise human PrP restored the interaction.

AFIP Diagnosis: Spleen: Lymphoid depletion and necrosis, diffuse, severe, with reticuloendothelial cell hyperplasia and eosinophilic to amphophilic intranuclear inclusion bodies, turkey, avian, etiology consistent with adenovirus. Conference Note: Hemorrhagic enteritis (HE) of turkeys, marble spleen disease (MSD) of pheasants, and avian adenovirus group II splenomegaly (AAS) of chickens are caused by type II adenoviruses. Experimental infections have demonstrated that HE virus will produce splenic lesions in chickens and pheasants; likewise, MSD and AAS virus isolates will infect turkeys. Lesions are often more pronounced in the duodenum; however, less severe lesions can occur in the proventriculus, gizzard, large intestine, cecum, cecal tonsils, and bursa of Fabricius. Periodic acid-Schiff (PAS) staining for glycogen reveals large granular aggregates of glycogen within many fibers and strong positive staining of the more organized aggregates visible on hematoxylin and eosin (H&E) stained sections. An enzyme defect is suspected in both the draft breeds and the quarter horse type breeds, but to date a specific enzyme defect has not been identified (Valberg et al.

Suspected IEM with Metabolic Acidosis, Diagnostic Flowchart The presence of metabolic acidosis is an important finding and the starting point for one of the two algorithms. Galactosemia and Lowe's syndrome (oculocerebrorenal syndrome, an X-linked recessive disorder with congenital cataracts, proximal RTA, and mental retardation) as well as many other metabolic disorders which present later are associated with a RTA, usually a proximal RTA. If these are normal (except for the changes found with lactic acidosis), then the lactate/pyruvate ratio and the glucose level will allow differentiation into 1) glycogen storage disease, gluconeogenesis disorders, or endocrine causes; 2) disorders of pyruvate metabolism; or 3) defects in mitochondrial energy metabolism. Suspected IEM with Neurologic Abnormalities and Without Metabolic Acidosis, Diagnostic Flowchart The neurologic abnormalities in the absence of metabolic acidosis is the starting point for the second major algorithm. Increased ammonia in the absence of acidosis is either due to a urea cycle defect or transient hyperammonemia of the newborn (THAN, a disorder of unknown etiology, more frequent in prematures, associated with profound hyperammonemia and coma). Fatty acid oxidation disorders, glutaric acidemia type II - patients may have dysmorphic features similar to Zellweger's, hypertrophic cardiomyopathy, and multicystic dysplastic kidneys.

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My research involves the use of x-ray crystallography, organic chemistry, and molecular biology to study the structure and function of proteins involved in disease and cell signaling. It has a number of interesting biological properties, including antiviral activity, inhibition of protein synthesis, initiation of apoptosis (programmed cell death), and ability to arrest the cell cycle.  It has potential as an anticancer and immunosuppressive drug, and is currently in Phase II clinical trials with the National Cancer Institute against a variety of human cancers and leukemias. PPT was originally isolated for its ability to remove palmitate from palmitoylated H-Ras; however, recent results indicate that PPT is targeted to the lysosome and therefore Ras may not be the the enzyme's natural target. Very little is known about PPT, and there is no structural information. We are determining the crystal structure of PPT with didemnin B bound to gain insight into the structure and mechanism of PPT and determine the mode of binding and inhibition by didemnin B. This information will be useful in learning more about the role of PPT in cellular signaling, and how didemnin B can disrupt the cell cycle. I am also interested in the structure and function of modular protein domains involved in cell signaling events and molecular recognition, particularly the PH (pleckstrin homology) and DH (Dbl homology) domains.

About the cycle and the microbes involved, the following are pertinent A. Methanogens are anaerobes B. Methylotrophs are aerobes C. Methanogens fix CO (sub) 2 to form acetate D. All are true 3. In a ruminant - A. Methane is a source of food B. Methane is an electron sink C. Methane is not involved D. Methane is toxic 4. Transduction functions best with a A. Lysogenic virus B. Lytic virus C. Cell to cell contact D. Pili 15. The lymphocyte coated with antibody involved in antibody production A. T (sub) H cell B. T (sub) C cell C. T (sub) S cell D. None of the above 40. A non-specific cell involved in removing foreign material from the body A. Phagocyte B. B cell C. Plasma cell D. Memory cell 41. A macrophage involved with the recognition and processing of an epitope would have A. Lysosome and MHC class II antigen B. Lysosome and MHC class I antigen C. Lysosome but not MHC D. Phagosome but not Lysosome 42.

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aerobe: an organism that uses oxygen as its terminal electron acceptor aerosol- suspension of particles in airborne water droplets; smaller particles (< 10 um in diameter) not only remain suspended in the air for longer, but are more likely to reach sites in the respiratory tract where they can initiate an infection AIDS: a progressive debilitating disease, usually fatal, resulting from dysfunctioning of the human immune immune system due to infection with the human immunodeficiency virus - HIV. cell wall- external cover that confers rigidity, shape, and antigenicity to bacteria and yeasts cellulitis- inflammation of connective tissue chemotaxis- movement of a cell in response to a concentration gradient chickenpox: disease caused by varicella zoster. GAS- Group A Streptococcus pyogenes gram-negative- a prokaryote whose cell wall contains an outer membrane containing lipopolysaccharide; relatively little peptidoglycan is present gram-positive- a prokaryote whose cell wall consists largely of peptidoglycan; an outer membrane is absent Gram's stain- a differential staining procedure H antigen- flagellin hemolysin- protein that lyses erythrocytes by causing pore formation in the membrane herpes simplex: hepatitis: Human Immunodeficiency Virus: the retrovirus that has been directly identified as a causative agent of AIDS. hyaluronic acid- mucopolysaccharide in extracellular substance; also present in capsule of S. pyogenes hyaluronidase- enzyme that degrades hyaluronic acid iatrogenic disease- illness induced by a medical procedure immunity, acquired: immunity that adapts after exposure to antigen; characterixed by T cells and macrophages. immunity, innate: immunity, cellular: immunity conferred by antigen-specific T cells and macrophages. Salmonella- genus of Gram-negative bacteria associated with food-borne disease scarlet fever- rash caused by Erythrogenic toxin Semmelweis -Austro-Hungarian 19th century physician who deduced that streptococcal sepsis occurring after childbirth was an iatrogenic illness sepsis :presence of bacteria in the bloodstream septicemia: a condition where bacteria are found in the blood which is normally sterile.

Lysosomal Diseases research Unit. I was employed as a research associate in the Department of Clinical Immunology following the appointment of my PhD. Since that time, I have worked in the Department of Chemical Pathology at the Women's and Children's Hospital and have focused my research studies in the area of the biochemistry of genetic diseases, using immunochemistry and cell biology techniques. I am currently the head of the Immunochemistry-Cell Biology section of the Lysosomal Diseases Research Unit in the Department of Chemical Pathology at the Women's and Children's Hospital and am a chief investigator on an NH&MRC program grant entitled 'Lysosomal storage disorders: diagnosis, treatment and lysosomal function'. My current research interests include the role of protein processing in genetic disease, immune responses to enzyme replacement therapy in the treatment of patients with lysosomal storage disorders and the cell biology of lysosomes. Antibody responses to enzyme infusion are being investigated with regard to the potential detrimental effects of generating antibody to the replacement protein (ie. incorrect targeting, enzyme inactivation, intracellular degradation) and the intracellular fate of antibody bound enzyme (ie.

You study the four cells which result from these divisions and you find a. in all four of the cells one of the strands of DNA would contain only 15N and the other would contain only 14N. b. in two of the cells both DNA strands would contain only 15N and in the other two cells, both DNA strands contain only 14N. c. in all four of the cells, each DNA strand (each single DNA strand) contains a mixture of 14N and 15N. d. in two of the cells both DNA strands contain only 14N and in the other two cells, one of the DNA strands (one strand of a double-stranded DNA molecule) contains only 14N and the other contains only 15N. 8. a. cytoplasmic matrix, bound ribosome, inside rough ER, ER vesicle, Golgi, lysosome, phagosome (digestosome) b. cytoplasmic matrix, free ribosome, smooth ER, nuclear envelope, Golgi, phagosome (digestosome) c. cytoplasmic matrix, bound ribosome, Golgi, Golgi vesicle, rough ER, phagosome (digestosome) d. cytoplasmic matrix, bound ribosome, rough ER, cytoplasmic matrix, Golgi, cytoplasmic matrix, lysosome, phagosome (digestosome) 15. In some cell, which doesn't have the ability to do fermentation, you are able to prevent the ATP synthetase molecules in the mitochondrial inner membrane from making ATP. In the cell where this has happened a. the energy normally trapped in ATP would be lost as heat but the cell could still make some ATP by coupled reactions in glycolysis and the Krebs cycle. b. are broken into fragments, the fragments are converted into glucose, glucose is broken down in glycolysis or in glycolysis and aerobic respiration and energy released is used to synthesize ATP. The blastopore of a vertebrate embryo a. is the only cell which does not have the same genes as all the rest of the cells in embryo.

The cells in the lag phase of a batch culture are A. newly added to the culture medium B. synthesizing needed enzymes C. metabolically active D. not actively dividing E. all of the above 8. After 5 hours in stationary phase the culture still has 108cells/ml. The cells that have specific receptors for antigen are A. monocytes B. polymorphonuclear leukocytes C. lymphocytes D. red blood cells 36. When a B cell is stimulated by antigen it differentiates into A. antibody producing cells B. memory cells C. plasma cells D. all of the above 38. Immunological memory is based on A. movement to the brain of cells that recognize the antigen B. multiplication of all lymphocytes during illness or immunization C. movement of cells that recognize the antigen to lymph nodes D.. multiplication of lymphocytes that recognize the antigen 39. Activation of macrophages is brought about by A. antigen stimulated B cells B. antigen stimulated T cells C. antibodies D. complement 40.

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In eukaryotes, cycloheximide inhibits protein synthesis in the cytoplasm, and chloramphenicol inhibits protein synthesis in the mitochondria. Acetylcholine Receptor Protein is a major transmembrane protein in neurons (nerve cells). Inside a cell, the length of microtubules can be adjusted according to the needs of the cell by adding or removing the above mentioned protein `monomers' to or from existing microtubules. In the next step, you incubate your microtubules and fluorescent labelled protein under conditions that favor microtubule assembly. c) Colchicine which is once used as an anticancer drug is found to bind the above mentioned free protein `monomers' with high affinity and prevent their assembly into new microtubules. Even though colchicine have no affinity to already formed microtubules, it is observed that when cells are treated with colchicine all the microtubules inside the cell disintegrate.

lactic acid bacteria A group of gram-positive, non-sporing bacteria which carry out a lactic acid fermentation of sugars. lactic acid fermentation A type of fermentation carried out by lactic acid bafcteria in which sugar (e.g. lactose, glucose, pentose) are converted either entirely (or almost entirely) to lactic acid (homolactic fermentation) or to a mixture of lactic acid and other products (heterolactic fermentation). latent virus A virus present in a cell, yet not causing any detectable effect. Lyme disease was named after a place called Lyme in Connecticut, USA, where this disease was first recognized and has rapidly become the most prevalent tick-borne disease in the United States. If you are uncertain of the spelling, use the alphabetic index to help you browse around the glossary, chances are the word is somewhere. The word may not be in the glossary, you can either suggest the word to me or help me define the word.

"We are at the beginning of being able to develop a permanent solution to problems associated with Gaucher disease," said John Barranger, M.D., Ph.D., leader of the research team and professor in the departments of pediatrics, human genetics and molecular genetics and biochemistry at the University of Pittsburgh. "Several years ago, we developed the first treatment for the disease using an enzyme that we engineered to enhance its delivery to cells derived from the bone marrow. This substance increases production of specialized white blood cells (hematopoietic stem cells) which are primitive cells that have the unique ability to reconstitute the entire bone marrow. The stem cells are removed from the blood during a procedure called leukapheresis and then exposed to a retrovirus that carries the corrective gene for GC deficiency. "We are studying how well these cells will compete with the existing marrow, where they will continue to divide and produce glucocerebrosidase. Other members of the research team include Paul Robbins, Ph.D., Sallie Boggs, Ph.D., Edward Ball, M.D., Maya Nimgaonkar, M.D., Erin Rice, M.S., Joseph Kiss, M.D., and Alfred Bahnson, Ph.D # # # MEDIA CONTACT: Lauren Ward TELE: (412) 624-2607 FAX: (412) 624-3184 EMAIL: WARDLA@A1.ISD.UPMC.EDU For more info, please see Genetics and Gene Therapy Archives.

In addition to the research themes described below, our group is also involved in gene discovery (linkage and positional cloning), the development of new methods for the DNA-based diagnosis of genetic diseases, regulation of gene expression and gene therapy. Basic studies include investigation of the evolutionary and structure/function relationships of lysosomal enzymes which are responsible for their unique properties including 1) targeted transport to the lysosome, 2) catalysis at acid pH and 3) lysosomal stability (i.e., they do not degrade each other). A particular focus is the nature of the genetic and biochemical lesions in patients with various lysosomal storage diseases (e.g., Fabry, Schindler, Maroteaux-Lamy, Farbers, and Nieman-Pick diseases) are under study in order to identify human mutations which will provide insight into the structure and function of these catabolic glycoproteins. To facilitate these studies, our laboratory has purified several human lysosomal enzymes, determined their amino acid sequences by microsequencing, and then isolated and sequenced their cDNA and genomic sequences, (i.e., alpha-galactosidase A, alpha-N-acetylgalactosaminidase, arylsulfatase B, ceramidase and sphingomyelinase). Molecular studies are also underway using a variety of techniques to determine the nature of the genetic lesions (e.g., insertions, deletions, point mutations) causing the deficient function of each of these enzymes in their respective lysosomal diseases. For other diseases in which gene replacement is feasible, efforts are being directed to insert the cDNA into retroviral vectors for gene transfer studies.

Still in its very early stages, Dr Frances Platt and Dr Terry Butters, explain how it works: For the past few years we have been studying the activities of a substance called NB-DNJ, trying to understand why it inhibits the replication of certain viruses. New Activity of the Drug, NB-DNJ In the course of our experiments, we made the unexpected observation that NB-DNJ inhibits an important cellular enzyme. The second is gene therapy, which aims to introduce a copy of the normal gene into the patient which will lead to the production of the normal enzyme and correct the enzyme deficiency. NB-DNJ and the Prevention of Lysosomal Storage Over the last two years we have been investigating the therapeutic potential of NB-DNJ in three separate ways. We are now studying cells derived from patients suffering from Gauchers disease and Tay-Sachs disease (kindly provided by Dr Bryan Winchester, Institute of Child Health, London) to see if we can prevent accumulation of glycolipids with NB-DNJ treatment, and to see if NB-DNJ treated cells can degrade the material which has already been stored within the lysosome. Identification of a Second Drug We have recently identified a second, more selective drug called NB-DGJ (which is very similar to NB-DNJ).

Research in the laboratory of Cell Pharmacology For a review on lysosome click here Lysosomes are the organelles in which biopolymeric material arising either from endocytosis or autophagy is degraded. They contain a large number of hydrolytic enzymes which acting either alone or sequentially can fully digest a wide range of macromolecules. Many human diseases afflicting children result from defects of the lysosomal enzymes or malfunctioning of the lysosomal membrane. The projects of the Cell Pharmacology laboratory are aimed at a fuller understanding of how lysosomes function and their role in cell physiology. Four of the projects have to do with the cathepsins, a family of lysosomal enzymes that digest proteins, while another investigates the nutritional role of protein digestion by lysosomes. A fourth project is concerned with the permeability properties of the lysosome membrane.

Glycerolipids - membrane constituents (except TAG) - made from phosphatidic acid Plasmaologens - Alpha,beta cis unsaturated, ether linked Fas- cardiac and central nervous system Cholesterol biosynthesis - C2 (actate) ->C5 isoprene ->C30 Squalene Limiting step: HMG CoA reductase: HMG-CoA-> 3R-mevalonate (precoursor to C5 compound) All contain all type of lipids, amounts vary: Lipase eats phospholipids, leaves cholesterol, so cholesterol is enriched in LDLs.

ISSN: 1052-5378 United States Department of Agriculture National Agricultural Library 10301 Baltimore Blvd. Beltsville, Maryland 20705-2351 Animal Models in Biomedical Research: Poultry SRB 92-17 Special Reference Briefs Series Animal Models in Biomedical Research: Poultry SRB 92-17 Quick Bibliography Series Cynthia Petrie Smith Animal Welfare Information Center September 1992 National Agricultural Library Cataloging Record: Smith, Cynthia Petrie Animal models in biomedical research : poultry. BOOKS AND PROCEEDINGS 001 Atlas of Avian Radiographic Anatomy S.A. Smith and B.J. Smith, Philadelphia: Saunders, c1992, 226 p. NAL call number: SF767 B57S65 1992 Descriptors: atlas, plates, xerordiaograph, avian medicine, integument, axial skeleton, appendicular skeleton, digestive, respiratory, urinary, and reproductive system, parrot, cockatiel, budgerigar, finch, toucan, pigeon, hawk, owl, duck, quail, gull. ), Proceedings from SCAW conference, Agriculture Animals in Research, September 6-7, 1990, Washington, DC, Scientists Center for Animal Welfare, February 1992, 112 p. NAL call number: HV4704 W38 1990 Descriptors: regulatory perspectives, AAALAC accreditation, surgery, welfare, behavior, transport, handling, restraint, cattle, equine, poultry, sheep, swine. Subcommittee on Poultry Nutrition, Committee on Animal Nutrition, Board on Agriculture, National Research Council (8th edition) National Research Council (U.S.), Subcommittee on Poultry Nutrition, Washington, DC; National Academy of Sciences, 1984, 71 p. NAL call number: SF494 N37 1984 Descriptors: energy, protein, amino acids, fats, minerals, vitamins, water, requirements for chickens, turkeys, geese, ducks, pheasants, and quail. 023 The Behaviour of the Domestic Fowl D.G.M. Wood-Gush, Heinemann Educational Books Ltd., London, 1971, 147 p. (ISBN 0-435-62920-4) NAL call number: QL785.5 P6W66 Descriptors: senses and perception, communication, displays, agonistic behavior, social behavior, mating behavior, maintenance behavior, hormones, feeding, drinking, and grooming behavior, learning, stress. (ISSN 0889-4434) NAL call number: QL698 C7 Descriptors: review articles, scope covers nutrition, endocrinology, infectious diseases, biochemistry of muscle and bone, genetics, poultry processing, anatomy, embryology, toxicology.

EM A large secondary lysosome is shown. The dark granular material is the acid phosphatase reaction product, and the less dense surrounding material is Hb. This process is seen in a type of liver damage called hepatic hemosiderosis, which is developed by some patients after repeated blood transfusions. It probably is the result of incorporation of large amounts of Hb and its iron residues into secondary lysosomes. Hemosiderosis is seen also in patients with chronic hemolytic anemia in which red blood cells are broken up in abnormally large numbers, releasing their Hb content.

Ardell, Jeffrey L, PhD.  Role of Cardiac nerve plexus in cardiac regulation.  University of South Alabama, Mobile, AL. Critz, Stuart D, PhD.  Role of kATP channel modulation in preconditioning.  Univ of South Alabama, Mobile, Mobile, AL. Li, Ming, PhD.  Functional regulation of cardiac sodium channels by voltage-dependent phosphorylation with protein kinase A.  Univ of South Alabama, Mobile, Mobile, AL. Bistrup, Annette.  Sulfotransferases involved in acute and chronic inflamation.  Univ of CA, San Francisco, San Francisco, CA. Burch, Grant H, MD.  The role of tenascin-x incardiac and muscle development.  Univ of CA, San Francisco, San Francisco, CA. Cooper, Douglas N, PhD.  Regulation of vasclar smooth muscle migration by Galectin-1.  Univ of CA, San Francisco, San Francisco, CA.

To understand the role of sequence motifs and chaperones in the targeting process. ER for secretion signal peptide no Nucleus 8-12 a.a.s with many Arg, Lys no Lysosome mannose-6-phosphate yes ER retention -KDEL at C-terminus yes Mitochondria mitochondrial signal peptide no II. Mechanism a. signal recognition particle (SRP) binds to signal sequence on the polypeptide b. translation is arrested c. SRP binds to the SRP receptor d. GTP is hydrolyzed, SRP is released e. translation resumes, protein translocates f. signal peptide is cleaved by signal peptidase B. Mitochondrial targeting 1. Components required a. mitochondrial signal peptide motif on protein i. signal peptide is an amphipathic helix ii. basic residues on one side of the helix b. cytosolic and mitochondrial hsp70s (chaperones) c. mitochondrial signal peptide receptor d. mitochondrial translocation pore e. ATP 2. Golgi to secretory vesicle a. proteins are sorted in TGN (trans golgi network) i. packaged one protein type per vesicle b. constitutive secretion i. secretion of vesicles without signal c. regulated secretion i. secretory vesicles are packaged and wait in cytosol for a signal B. Lysosomal targeting 1.

The following terms will be used to compile an index for the abstract book. Please type the numbers corresponding to a maximum of three keywords at the bottom of your abstract and outside the main body of the text . For example, if your abstract describes a novel chaperone machine from archaebacteria with considerable sequence homology to known proteins your keyword list might be 2, 8, 40h. If you believe that a term not included on the current list would more accurately describe the contents of your abstract please type the actual term; it should be possible to include additional terms in future indexes. Oxidative stress

This page last updated January 15, 1995. Area of general research interest: Role of Lipoproteins in Atherogenesis Current program: Mechanisms responsible for formation of lipid-protein cross-links in cells that have internalized oxidized LDL. Biological and clinical properties of Lp(a) and LDL in which arachidonic acid has been oxidized. Cellular recognition of novel epitopes formed on LDL during oxidation.

glucocerebrosidase Construct pPNT-MGC2 was made by subcloning a 2,797-bp Sspl-SalI fragment containing all of exons 5 to 8 and part of exon 9 of the murine glucocerebrosidase ge. glucocerebrosidase Construct pPNT-MGC2 was made by subcloning a 2,797-bp Sspl-SalI fragment containing all of exons 5 to 8 and part of exon 9 of the murine glucocerebrosidase ge. DR PRODOM [Domain structure / List of seq.

W.F. (Bill) Carey was awarded the degree of Doctor of Philosophy in Biochemistry by the University of Adelaide in 1971. His accomplishments include establishing many of the enzyme assays and other biochemical protocols used routinely to diagnose patients with various inherited disorders. His current interests are in determining molecular explanations for variation of clinical expression within genetic disease and he is co-recipient of National Health and Reseach Council of Australia funding to investigate the molecular variability in metachromatic leucodystrophy. He is currently Head of the National Referral Laboratory which provides a prenatal and postnatal diagnostic service for a wide range of biochemically defined genetic disorders to Australia, New Zealand and South East AsiaWith Dr. C.Phil Morris (who moved to the Quensland University of Technology in April, 1996), Bill currently holds a National Health and Research Council grant to investigate the molecular biology of metachromatic leucodystrophy (MLD). The expressed ASA gene has been engineered to contain a various combinations of polymorphisms, each by themselves shown not to reduce the level of ASA activity to levels hypothesised to cause disease.

There are many sites on the World Wide Web that have information about specific diseases. The Down Syndrome WWW Page has information about Welcoming a Down Syndrome Baby, and lots of information about where to find more information. Here are a few sites that have lists and lists of sites that will lead you to specific information about other diseases. At this site you'll find lots of information about children's conditions and diseases. The National Health Information Center (NHIC) lists a series of categories under which there is health information and referral services. As with any advice or information you might find, check it out with people you trust and visit with your doctor before taking any action that might affect your or your family's health!

STRUCTURE: A lysosome is a special type of vesicle containing hydrolytic enzymes. The lysosomes itself is a membrane-enclosed sac. FUNCTION: Lysosomes, due to their enzymes, are used to digest (or hydrolyze) various macromolecules. Lysosomes play a crucial role in phagocytosis. The lysosome then fuses to the vacuole, and the enzymes of the lysosome digest the contents of the vacuole. In order for lysosomes to function properly, an acidic envir onment is needed.

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Because the familial form is an autosomal recessive disorder, people can be heterozygotes and thus "carriers" of TSD. Carriers have no symptoms of the disease, and consequently could be totally ignorant of their status until they are tested or have a child who has TSD. A man and woman who are both carriers have a 25 percent chance of having a child with TSD, and a 50 percent chance of having a child who is a carrier. While the incidence of TSD heterozygotes in most Jews and non-Jews is 0.2 percent, it has been estimated to be as high as 4 percent[2] in the Ashkenazi Jewish population. Their general unfamiliarity with TSD was not unexpected; eighty-nine percent had never attended a course, lecture, or workshop about TSD. Ninety-six percent of those who had never distributed literature about TSD said they would if such materials were available to them. As part of a study of high school views of human genetics and TSD, Clow and Scriver (1977) investigated 45 TSD carriers and 45 non-carriers who had been screened in a Montreal high school program. It seems that positive carrier status had no adverse effects on relationships in comparison to non-carriers; 32 percent of the carriers and 16 percent of the noncarriers were engaged. The group seemed to find heterozygosity a greater obstacle for others than for themselves; although 73 percent thought that other carriers might change their marriage plans if their partner were found to be a carrier, only 3 percent said they would themselves.

Exam III to Final - Fall 1996 Review Session: 2-4 p.m., Wednesday and Thursday (December 11, 12) 4524 Gardner Hall Hoatzin Honey Guide Syntrophy "Normal" flora Mouth Respiratory System GI tract GU tract Physical barriers to infection Chemical defenses Inflammatory response Phagocytes PMNs Macrophages/monocytes Lysosome Phagosome Phagolysosome-enzymes Pathogenesis mechanisms Attachment or adherence Colonization and virulence Toxin production Exotoxin/endotoxin Plasma cell Memory cell Complement Epidemic Endemic Pandemic Incidence of disease Mortality rate Morbidity rate Carrier Reservoir "Typhoid Mary" Vaccination Down side immune response Anaphylactic shock Allergy Autoimmune Antigen/Immunogen Basic properties immune response Epitope Effective Antigens Immunoglobulin (Ig) IgA, G, M, D, E Constant region Variable region Active/Passive Immunity B-cell role T-cell role MHC Type I MHC Type II CD4+ cell CD8+ cell T-cell antigen receptor How many different B-cells Contact disease Food and water borne STD's Vector borne Nosocomial infection Herd community Influenza and cold Reyes syndrome HIV/AIDS Effect on CD4+ cells Diseases associated

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Phagocytes and Phagocytosis Distinct cell type arise from multipotent stem cells - bone marrow Phagocytic cells important - Immune response alone - not enough Defects in phagocytic cell production - constant infections Two major phagocytic cell types (1) Polymorphonuclear Leukocytes (PMNS) (2) Monocytes - macrophages PMNS Circulating leukocytes - remove debris including bacteria by engulfment Move rapidly - first at site of inflammation Short lived - present in large numbers response to infection Stimulated release from bone marrow - indicates active infection Sequence of events Phagocytosis (drawing) Phagosome contains bacterium - combines with lysosome Form phagolysosome releasing hydrolytic enzymes into phagosome - 60 enzyme systems in lysosome all destructors Proteases Lipases Carbohydrases RNAase DNAase acid phosphatase peroxidase Peroxide NADPH + O (sub) 2 + H (super) + - H (sub) 2 O (sub) 2 Superoxide NADPH + 20 (sub) 2 - O (sub) 2 (super) - + NADP Myeloperoxidasse Cl (super) - + H (sub) 2 O (sub) 2 - Clo (super) - + H (sub) 2 O hypochlorite Monocytes Macrophages are large cells - prevent invasion and involved with immune response Some move into tissue wandering macrophages Others limited movement - or fixed. Enzymes in phagolysosome are more limited than those in PMNS Lymphatic Network - vessels - ducts Carries macrophages Cover more immune response Pathogenesis mechanisms Organisms that cause disease Obligate - Neisseria gonorrhoeae Accidental - Clostridium tetani Opportunistic - weakened immune response - AIDS yeasts, fungi, pseudomonas How does disease process go - Adherence to selected tissue much disease originates - mucosal surfaces respiratory GI GU Some examples: Fimbriae - N gonorrhoeae - GU tract epithelia Salmonella - intestine epithelia Capsule - S mutans dextrans attach to teeth Colonization and Virulence Growth disruption of cells collagenase elastase hyaluronidase lecithinase Coagulase - staph - form clot to ward off phagocyte also can produce along with streptococci streptokinase - dissolve clots and penetrate further Virulence ability to cause disease measured by lethal dose 50 or LD50 number of microorganisms or agent that will kill 50% of population virulence = invasiveness + toxin production Mere presence of microbes rarely causes disease Clostridium tetani invasiveness - toxigenicity ++1++ fatal Streptococcus pneumoniae invasiveness +++++ toxigenicity +/- can be fatal Invasion - counteract host Phagocytes line of defense destroy Leukocidins - pneumococci Streptococci Staphylocci capsule of strept pneumoniae Brucella - undulant fever inhibits phagolysosome Carried about in phagosome Legionella can propagate in monocytes Once established - Harm - disease systems product of toxin production Endotoxins Exotoxins now divide into Entero, Neuro, etc.

PNAS Lipid thioesters derived from acylated proteins accumulate in infantile neuronal ceroid lipofuscinosis: Correction of the defect in lymphoblasts by recombinant palmitoyl-protein thioesterase Lipid thioesters derived from acylated proteins accumulate in infantile neuronal ceroid lipofuscinosis: Correction of the defect in lymphoblasts by recombinant palmitoyl-protein thioesterase

Nervous system damage in many acquired allergic and infectious demyelinating diseases is specifically directed against myelin or myelin-forming cells with relatively little damage to other parenchymal elements /773 Multiple sclerosis is the most common demyelinating disease of the central nervous system in man / 773 Biochemical analyses of MS lesions reveal an increase of catabolic enzymes and a severe loss of myelin proteins and lipids / 775 Although the cause of MS is unknown, genetic, immunological, and environmental factors are believed to contribute to its pathogenesis /776 Experimental allergic encephalomyelitis is an animal model of autoimmune demyelination / 778 Guillain-Barre$PI$aa syndrome is an acute, monophasic, inflammatory demyelinating disease of the peripheral nervous system, often preceded by a viral infection / 781 Carbohydrate epitopes on the myelin-associated glycoprotein and/or other glycoconjugates are targets for autoimmune demyelination of the peripheral nervous system occurring in association with paraproteinemia / 781 Other acquired demyelinating diseases in humans may be secondary to viral infections, neoplasia, or immunosuppressive therapy /782 A number of animal diseases caused by viruses involve primary demyelination and are often associated with inflammation / 783 Biological toxins that produce myelin loss can be produced by exogenous infectious agents (e.g., diphtheria toxin) or lymphocytes (cytokines) / 787 Organotin and hexachlorophene cause edematous demyelination with splitting at the intraperiod line and without apparent damage to myelin-forming cells / 787 Lead is a common environmental pollutant that causes hypomyelination and demyelination /788 Tellurium treatment of young rats causes a demyelinating neuropathy / 788 Undernourishment leads to a preferential reduction in myelin formation / 788 Dietary deficiencies of specific substances can cause myelin deficits / 788 The archetypical model for secondary demyelination is Wallerian degeneration /789 Secondary demyelination occurs in subacute sclerosing panencephalitis and other diseases of the central nervous system / 789 The capacity for remyelination is much greater in the peripheral nervous system than the central nervous system / 790 Remyelination in the central nervous system can be promoted by various treatments, and therapy of human myelin disorders by this approach may be feasible / 790 Carbamyl phosphate synthetase deficiency /834 N-Acetylglutamate synthetase deficiency /834 Ornithine transcarbamylase deficiency / 834 Citrullinemia / 834 Argininosuccinic aciduria / 835 Arginase deficiency / 835 Hyperornithinemia, hyperammonemia, homocitrullinuria syndrome / 835 Lysinuric protein intolerance / 835 Management of urea cycle defects / 835 Elevated ammonia produces severe CNS toxicity / 843 Abnormalities may involve neurotransmitter amino acids / 844 Abnormalities in protein synthesis are produced by ammonia and liver disease / 845 Elevated ammonia depresses metabolic energy reserves / 846

The ultrastructural neuropathology of mice experimentally inoculated with brain tissue of nyala (Tragelaphus angasi; subfamily Bovinae), or kudu (Tragelaphus strepsiceros; subfamily Bovinae) affected with spongiform encephalopathy was compared with that of mice inoculated with brain tissue from cows (Bos taurus; subfamily Bovinae) with bovine spongiform encephalopathy (BSE). The results show that the same strain of agent caused disease in the BSE cases, and that the relationship of BSE to scrapie in sheep is unclear. A total of 1453 such herds, comprising 166 603 adult animals, experienced at least one case of BSE with clinical onset between January 1 and June 30, 1989; 2481 herds, comprising 281 648 adult animals, experienced at least one case with clinical onset between January 1 and June 30, 1990 and 3334 herds, comprising 359 488 adult animals, experienced at least one case with clinical onset in the first 6 months of 1991. , En This publication consists of a number of appendices (presumably papers presented at the meeting) covering: review of the foot and mouth disease (FMD) situation in the world; report of meetings in Thailand and follow-up; FMD risk assessment; distribution of exotic FMD viruses; African horse sickness situation in Spain and Morocco; viral haemorrhagic disease of rabbits; review on bovine spongiform encephalopathy; joint meeting of the Code Commission; and revision of OIE list A diseases, horse health certificate, reference laboratories. Negative stain electron microscopy of proteinase K-treated detergent extracts of tissue from the brain stem revealed the presence of scrapie associated fibrils, and a 25 to 28 kDa band comparable with that identified as abnormal PrP (prion protein) from the brains of domestic cattle with spongiform encephalopathy was detected using rabbit antiserum raised against mouse PrP. The animal was born 9 months after the statutory ban on the inclusion of ruminant-derived protein in ruminant feeds and, as no other possible sources of the disease were apparent, it appears likely that the infection was acquired from the dam. Primary transmissions of natural BSE was attempted to cattle, hamsters, pigs and domestic fowl by parenteral inoculation of brain homogenate; to pigs and domestic fowl by oral/dietary exposure to homogenised brain and to cattle by oronasal exposure to homogenised fetal membranes collected in late gestation.

This is an incomplete collection of Summary Charts to aid the medical student in the subject of Microbiology Subjects include: Bacteria Fungus Parasites written by Aaron J. Geswaldo Summer 1996 UMDNJ-SOM STAPH AND STREP written by Aaron J. Geswaldo Toxic shock - syndrome toxin Direct Invasion/Toxin Pharyngitis Red, swollen tonsils and pharynx, purulent exudate on tonsils, Fever Swollen Lymph nodes Skin Infections Folliculilltis cellulitis, impetigo Scarlet Fever Toxic Shock syndrome Antibody Mediated Rheumatic Fever (may follow streptococcal pharyngitis) Fever Myocarditis Arthritis, chorea Rash Subcutaneous Nodules 10 - 20 years after infection, may develop permanent heart valve damage Acute post-streptococcal glomerulonephritis tea-colored urine, following streptococcal skin or pharynx infection Penicillin G, V Erythromycin Following rheumatic fever - patients are placed on continuous prophylactic antibiotics to prevent repeat strep throat infection, that could potentially cause a repeat case of rheumatic fever If permanent heart valve damage has occurred, antibiotics are required when certain procedures are performed (such as dental work), due to the high risk of bacterial endocarditis. Catalase-negative Facultative anaerobe Beta hemolytic Neonatal meningitis Neonatal pneumonia Neonatal sepsis Penicillin G Part of the normal flora - 25% of pregnant women carry group B streptococci in their vagina Lancefield group D Two sub types Catalase-negative Facultative anaerobe Alpha, beta, or gamma - hemolytic Subacute bacterial endocarditis Billary tract infections Urinary tract infections Ampicillin sometimes combined with an aminoglycoside Resistant to penicillin G Catalase-negative Facultative anaerobe Alpha-hemolytic Subacute bacterial endocarditis Dental caries - caused by streptococcus mutans Brain or liver abscesses - caused by streptococcus anginosa Penicillin G Part of the normal oral flora (founded in the nasopharynx and gingival cervices) Catalase-positive Coagulase-negative Facultative anaerobe Highly resistant to antibiotics nosocomial infections Prosthetic Joints Prosthetic heart valves Sepsis from intravenous lines Urinary tract infections Frequent skin contaminant in blood cultures Vancomycin

Lysosome-like structures Occasional smaller, lysosome-like structures (arrowheads) occur in the vicinity of the larger vesicles. Last modified: April 15, 1996 Claudia Sommer, sommer@rzbox.uni-wuerzburg.de

D4DR_HUMAN 3 +1 blaize 256-351 222-257 p(0.00822) D(4) DOPAMINE RECEPTOR (D(2C) DOPAMINE RECEPTOR). E75C_DROME 3 +1 blaize 172-354 230-288 p(0.006) ECDYSONE-INDUCIBLE PROTEIN E75-C. EBN4_EBV 3 +1 blaize 280-405 714-757 p(0.00822) EBNA-4 NUCLEAR PROTEIN (EBNA-3B). ENV_MLVFP 3 +1 blaize 46-183 248-292 p(0.0525) ENV POLYPROTEIN PRECURSOR (CONTAINS: KNOB PROTEIN GP70; SPIKE PROTEIN P15E; R PROTEIN). L37509 3 +3 tblastx 186-365 275-96 p(3.1e-05) Brassica rapa (clone F1133) expressed sequence tag (EST) gb| 976347 3 +1 blastx 115-162 2081-2096 p(0.00086) (L32832) zinc finger homeodomain protein [Homo sapiens]

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