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25 Cards in this Set

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Leukocyte adhesion deficiency II (LAD II)
-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
The characteristic bond through the anomeric carbon is called what?
Glycosidic linkage
How are mannose, glucose & galactose related?
stereoisomers of each other
Maltose Structure
two glucose in alpha 1-->4 linkage
Lactose Structure
galactose & glucose in beta 1-->4 linkage
Sucrose Structure
fructose & glucose
What is starch & what are the two forms & composition?
-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
What is glycogen & what is the composition?
-carbohydrate storage in animals

-homopolymer of glucose

-alpha 1-->4 linkages w/ alpha 1-->6 branches every 8-12 residues
What is the structural composition of cellulose?
-homopolymer of glucose

-unbranched with beta 1-->4 linkages
What are GAGs? Where are they found? What does their synthesis involve? What do they normally contain? What is an example of a GAG?
-long unbranched polysaccharides of repeating, anionic (neg. charged), disaccharides

-found in connective tissues

-synthesis involves activated sugar (UDP)

-normally contain sulfate

-ex. Hyaluronic acid
What is the name of a GAG chain covalently linked to a protein?
proteoglycan
Example of how proteins react chemically with carbohydrates?
-Glycated Hemoglobin (HbA1C) provides a "history" of glucose levels
Where do glycosylated proteins end up?
-glycosylation is for secreted proteins or domains facing extracellularly (or into lumen of ER)

-EXCEPTION OF O-GlcNac
Which residues do O-linked glycosylations link to? Where are they glycosylated? What enzyme adds the first sugars? What adds the rest?
-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
What residue do N-linked glycosylations link to? How are they added? Where does it happen?
-Asparagine

-Added first to dolichol phosphate (lipid carrier molecule) --> then transferred in tact to protein

-generally done co-translationally in the ER
What does tunicamycin do?
-selectively inhibits adddition of N-aacetylglucosamine-phosphate to dolichol phosphate
What happens to the glycosylations while the protein travels down from ER --> golgi? How do lysosomes break down glycosylations?
-they constantly are getting contents modified

-there are diseases associated with defective modifying enzymes

-lysosomes use endo & exoglycosidases
What do proteoglycans usually consist of?
-a core protein O-linked to a GAG
What are some examples of proteoglycans (3 of them)? Where can they be found?
-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
What are glycoproteins recognized by?
lectins
What are 5 important aspects of protein glycosylation recognition?
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
Why are blood type O individuals more prone to Ulcers?
-because H. Pylori binds specific structures that look like blood type O sugars
Blood type is determined by what?
-glycosylation at end of carb chains of glycoproteins or glycolipids
How did the bird flu kill so many people?
mutated to recognized 2-->6 linkage rather than 2-->3 linkage so could infect humans
What glycosylation sends proteins to the lysosome?
M6P