Glycogen structure and metabolism overview — Flashcards

Flashcard 1: Which glycogen storage disorder is associated with severe fasting hypoglycemia? _____

Answer: Von Gierke disease

Flashcard 2: Glycogen phosphorylase is regulated via positive feedback by _____ (liver)

Answer: glucagon

Flashcard 3: Dephosphorylation of glycogen synthase causes enzyme _____ (activation/inactivation)

Answer: activation

Flashcard 4: Glucokinase is regulated via positive feedback by _____

Answer: insulin

Flashcard 5: What is the rate-limiting enzyme for glycogenolysis? _____

Answer: Glycogen phosphorylase

Flashcard 6: In skeletal muscle, glycogenolysis ultimately produces _____, which is rapidly metabolized during exercise

Answer: glucose-6-phosphate

Flashcard 7: glycogen synthesis

Answer: glycogen synthase

Flashcard 8: glycogenolysis

Answer: glycogen phosphorylase

Flashcard 9: McArdle's disease (GSD type V)

Answer:

Extra: painful muscle cramps, myoglobinuria with strenuous exerciseglycogen accumulation in muscleARskeletal muscle glycogen phosphorylasemnemonic: Very Bad Carbohydrate Metabolism (the glycogen storage diseases in order)

Flashcard 10: Cori's disease (GSD type III)

Answer:

Extra: hepatomegalyfasting hypoglycemia, increased hepatic glycogen stores, normal blood lactate levelsARglycogen debranching enzymemnemonic: Very Bad Carbohydrate Metabolism (the glycogen storage diseases in order)