Glycogen Storage Diseases — Flashcards

Flashcard 1: Von Gierke disease is characterized by _____ gluconeogenesis and impaired glycogenolysis (impaired or functional)

Answer: impaired

Flashcard 2: The branched glycogen molecule is cleaved by the enzyme _____ in the lysosomes only.

Answer: α-1,4-glucosidase (acid maltase)

Flashcard 3: _____ disease is also known as glycogen storage disease type V

Answer: McArdle

Flashcard 4: Hers disease is characterized by _____ gluconeogenesis and impaired glycogenolysis (impaired or functional)

Answer: functional

Flashcard 5: _____ disease is caused by a deficiency of the enzyme muscle glycogen phosphorylase (myophosphorylase)

Answer: McArdle

Flashcard 6: _____ disease is also known as glycogen storage disease type II

Answer: Pompe

Flashcard 7: Which steps (2) of glycolysis require ATP? 1. _____ (Hexokinase/Glucokinase) 2. _____ (PFK-1)

Answer: Glucose -> Glucose-6-phosphate

Flashcard 8: _____ is the glycosaminoglycan present in the granules of the given mononuclear cell / mast cell.

Answer: Heparin

Flashcard 9: Which steps (2) of glycolysis produce ATP?

Answer: 1. 1,3-bisphosphoglycerate → 3-phosphoglycerate (catalyzed by phosphoglycerate kinase) 2. Phosphoenolpyruvate → pyruvate (catalyzed by pyruvate kinase) Note: Both of these are examples of substrate-level phosphorylation.

Flashcard 10: breast millk is richest in _____ carbohydrate

Answer: lactose

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