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14 Cards in this Set

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  • Back
What are the main stores of glycogen in the body?
Liver and muscle
What is the primary bond within a glycogen molecule?
Glycosidic α-1,4- linkage
When is muscle glycogen affected?
strenuous exercise or several week fast
Glycogen synthesis
In short: Y residue of Glycogenin is Glycosylated --> glycogen Synthase elongates chain by forming new α 1,4 linkages --> branching enzyme creates new α 1,6 linkages --> glycogen Synthase elongates branches
What is glycogenin and when do you need it?
a glycogen primer, and it is only needed when glycogen is completely depleted
how is UDP converted to UTP?
nucleoside diphosphokinase + ATP
coenzyme required for glycogen degradation?
pyridoxal phosphate
Name the enzyme that removes glucose from glycogen and where it cleaves.
Glycogen phosphorylase cleaves at α 1,4 glycosidic bonds at non-reducing ends
What is the limit dextrin?
Glycogen phosphorylase stops chopping off glucose when 4 glucosyl residues remain before each branch point
How are branches removed during glycogen degradation?
- Glucosyl 4,4 transferase removes outer 3 of 4 glucose residues and transfers them ot the non-reducing ends of another chain
- amylo-α 1,6 glucosidase breaks an α 1,6 bond releasing free glucose
- glycogen phosphorylase now goes to work again until next limit dextrin is reached
What does glucosyl 4,4 transferase do?
removes outer 3 of 4 glucosyl residues off the limit dextrin
What is the product of glycogen degradation?
glucose1p --> phosphoglucomutase --> glucose6P
What is phosphoglucomutase?
converts the glycogen degradation product glucose1P to glucose 6P the glycolytic molecule
What is Pompe's?
- lysosomal enzyme x,4