MSKS BioChem Lecture 3_Energy Metabolism in Muscle Part 2 Flashcards
What Hormonally activates and inhibits glycogen phosphoralase?
Activated by epinephrine and inhibited by insulin
What are the 6 glycogen storage diseases, their enzyme deficency, their clinical manifestations, and the structure of the associated glycogen?
What is the role of Glycogenin?
‣It is a protein that serves as a primer ‣Tyr is an attachment point for UDP-glucose ‣Glycogenin has catalytic activity
What is the rate limiting step of Glycogenesis
glycogen synthase bonding UDP glucose to the glycogen chain
What are the three forms of Creatine kinase and where are they found?
- CK1 = BB, found only in brain
- CK2 = MB, found only in heart
- CK3 = MM, found in skeletal muscle and heart
What Hormonally activates and inhibits glycogen synthase?
Activated by insulin and inhibited by epinepherine
What allostericly activates and inhibits glycogen synthase?
It is allosterically activated by glucose 6 phosphate. It is not directly allosterically inhibited by anything
How does Epinephrine upregulate glucose levels in a myofiber?
It activates the adenelyl cyclase pathway. The pathway ultimetly phosphoralates glucose phosphoralase thus converting it into its active form. Additionally, protein kinase A phosporlates glycogen synthase thus converting it into its inactive form. Insulin does the exact opposite although via a different pathway.
How does Ca increase glycogenolysis?
Ca binds to the calmoduin of Phosphoralase kinase and activates it without phosphoralation. Phosphoralase kinase then activates Glycogen phosphoralase.
What allostericly activates and inhibits glycogen phosphoralase?
It is allosterically activated by Ca and AMP. It is allosterically inhibitied by ATP and glucose 6 phosphate.
What causes Pompe Disease and what does it cause?
