Glycogen storage diseases — Flashcards

Flashcard 1: glycogen synthesis

Answer: glycogen synthase

Flashcard 2: glycogenolysis

Answer: glycogen phosphorylase

Flashcard 3: 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 4: 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)

Flashcard 5: von Gierke's disease (GSD type I)

Answer:

Extra: hepatomegalysevere fasting hypoglycemia, increased blood lactatevery increased hepatic glycogen storesARglucose-6-phosphatasemnemonic: Very Bad Carbohydrate Metabolism (the glycogen storage diseases in order)

Flashcard 6: Describe, in general, the glycogen storage diseases.

Answer: Diverse group of diseases resulting from defects in glycogen metabolism, characterized by an accumulation of glycogen within cells.

Flashcard 7: Describe the glycogen molecule structure.

Answer: Large, branched polymer of glucose

Flashcard 8: What are the main sites of glycogen storage?

Answer: liver, muscle

Flashcard 9: What type of bonds are found at glycogen linkages?

Answer: α (1,4) bonds

Flashcard 10: What type of bonds are found at glycogen branches?

Answer: α (1,6) bonds