Carbs IV Flashcards
Where is glycogen synthesis performed?
muscle and liver
What is the difference between storage location of glycogen?
muscle = stores only for muscle
liver = stores for entire body
What is the function of UDP-glucose pyrophosphorylase?
to form UDP-glucose from UTP and G1P
What is the function of phosphoglucomutase?
G6P to G1P
Glycogen phosphorylase releases glucose molecules from glycogen in what form?
G1P
Is de-branching enzyme efficient?
one glucose is lost as G1P due to alpha-glucosidase activity
What product inhibits the activity of hexokinase?
G6P
What is the difference between muscle and liver in response to glucagon stimulation?
muscles do not possess glucagon receptors
What other property of muscles contributes to their inability to respond to glucagon?
muscles do not possess glucose-6-phosphatase
What is the difference between muscle and liver and their glycogenolysis reactions in response to epinephrine?
epinephrine causes the liver the breakdown glycogen
epinephrine causes the muscle to breakdown glycogen and to also increase glycolysis
What are the effetcs of PKA on glycogen synthase?
glycogen synthase is inactivated by PKA
What do patients with a glycogen storage disease usually present with?
muscle weakness/exercise intolerance
enlarged liver
What defect is present in Van Gierke Disease? (type I)
defect in glucose-6-phosphatase
What defect is present in Pompe Disease? (type II)
alpha-glucosidase deficiency
lysosomes fill with glycogen
What defect is present in Cori diease? (type III)
glycogen debranching enzyme