Glycogen Flashcards
Where is glycogen stored
the liver and muscle
what is the rate limiting step in producing glycogen
glycogen synthase converting UDP-glucose to glycogen alpha 1-4
what does glycogenin do
acts as a primer for glycogen synthase
what enzyme takes alpha 1-4 glucose and converts it to alpha 1-6 glucose and what does it produce in the process
branching enzyme, one free glucose molecule
what can’t Glucose 6 phosphate be broken down via glycolysis in the liver
because the liver doesn’t have any F-2,6-BP so PFK-1 doesn’t work
Where is glycogen phosphorylase A found
liver (more active)
where is glycogen phosphorylase b found
muscle
what is phosphorylase b activated by and inactivated by
Ca++ and AMP.
ATP and G6P
What is phosphorylase a activated by and inactivated by
Activated by nothing and inactivated by ATP and g6p
what is the pathway for glucagon signal transduction
glucagon activates Gprotein–>adenylyl cyclase–cAMP–>PKA–>deactivates phosphorylase kinase (so phosphorylase b is active)
What is type I GSD
Von Gierke, defective glucose phosphatase
What is type II GSD
Pompe, propblem with alpha 1-4 glucosidase, polysaccharides can’t be broken down
what is type III GSD
Cori, oddly structured glycogen with short arms
what is type IV GSD
Andersen’s, oddly structured glycogen, long arms
What is type V GSD
MacArdle, muscle phosphorylase deficience. Lactate does not increase during exeercise
