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25 Cards in this Set
- Front
- Back
Leukocyte adhesion deficiency II (LAD II)
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-rare autosomal recessive syndrome associated with Bombay (hh) blood type
-patients have frequent infections b/c of defect in fucosylation --> white cells don't go where supposed to effectivey -cell to cell contact mediated by sugar --> necessary for blood cells to slow down & dock -MUTUAL INTERACTION B/W ENDOTHELIAL CELLS & WHITE BLOOD CELLS, both have receptors |
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The characteristic bond through the anomeric carbon is called what?
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Glycosidic linkage
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How are mannose, glucose & galactose related?
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stereoisomers of each other
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Maltose Structure
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two glucose in alpha 1-->4 linkage
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Lactose Structure
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galactose & glucose in beta 1-->4 linkage
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Sucrose Structure
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fructose & glucose
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What is starch & what are the two forms & composition?
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-storage form of carb in plant
-both homopolymers of glucose 1) Amylose: unbranced chain with alpha 1-->4 linkages 2) amylopectin: alpha 1-->4 linkages w/ alpha 1-->6 branches every 12 residues |
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What is glycogen & what is the composition?
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-carbohydrate storage in animals
-homopolymer of glucose -alpha 1-->4 linkages w/ alpha 1-->6 branches every 8-12 residues |
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What is the structural composition of cellulose?
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-homopolymer of glucose
-unbranched with beta 1-->4 linkages |
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What are GAGs? Where are they found? What does their synthesis involve? What do they normally contain? What is an example of a GAG?
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-long unbranched polysaccharides of repeating, anionic (neg. charged), disaccharides
-found in connective tissues -synthesis involves activated sugar (UDP) -normally contain sulfate -ex. Hyaluronic acid |
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What is the name of a GAG chain covalently linked to a protein?
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proteoglycan
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Example of how proteins react chemically with carbohydrates?
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-Glycated Hemoglobin (HbA1C) provides a "history" of glucose levels
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Where do glycosylated proteins end up?
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-glycosylation is for secreted proteins or domains facing extracellularly (or into lumen of ER)
-EXCEPTION OF O-GlcNac |
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Which residues do O-linked glycosylations link to? Where are they glycosylated? What enzyme adds the first sugars? What adds the rest?
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-Serine or Threonine
-glycosylated in Golgi -first sugar: N-acetylgalactosaminyl-transferase -rest of sugars: glycotransferases --> SPECIFIC ENZYME FOR EACH STOP OF SYNTHESIS -ONE AT A TIME PROCESS & add it directly to protein |
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What residue do N-linked glycosylations link to? How are they added? Where does it happen?
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-Asparagine
-Added first to dolichol phosphate (lipid carrier molecule) --> then transferred in tact to protein -generally done co-translationally in the ER |
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What does tunicamycin do?
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-selectively inhibits adddition of N-aacetylglucosamine-phosphate to dolichol phosphate
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What happens to the glycosylations while the protein travels down from ER --> golgi? How do lysosomes break down glycosylations?
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-they constantly are getting contents modified
-there are diseases associated with defective modifying enzymes -lysosomes use endo & exoglycosidases |
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What do proteoglycans usually consist of?
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-a core protein O-linked to a GAG
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What are some examples of proteoglycans (3 of them)? Where can they be found?
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-chondroitin sulfate
-heparin sulfate (the one that binds antithrombin to thrombin) -keratan sulfate -can be found in basement membrane, in knee cartilage acting as shock absorber |
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What are glycoproteins recognized by?
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lectins
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What are 5 important aspects of protein glycosylation recognition?
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1) assist protein folding in ER
2) intracellular proteins sorting/transport 3) first line of defense in immunity 4) determine half-life of serum proteins 5) direct cellular trafficking in vascular system |
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Why are blood type O individuals more prone to Ulcers?
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-because H. Pylori binds specific structures that look like blood type O sugars
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Blood type is determined by what?
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-glycosylation at end of carb chains of glycoproteins or glycolipids
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How did the bird flu kill so many people?
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mutated to recognized 2-->6 linkage rather than 2-->3 linkage so could infect humans
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What glycosylation sends proteins to the lysosome?
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M6P
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