Disorders of Fructose and Galactose Metabolism — Flashcards

Flashcard 1: _____ is characterized by the absence of the Gal-1-P uridyltransferase enzyme

Answer: Classic galactosemia

Flashcard 2: Cori disease is characterized by _____ gluconeogenesis and impaired glycogenolysis (impaired or functional)

Answer: functional

Flashcard 3: Hers disease is characterized by _____ gluconeogenesis and impaired glycogenolysis (impaired or functional)

Answer: functional

Flashcard 4: Galactose-1-phosphate is ultimately converted to _____ via the enzyme Gal 1-P uridyltransferase

Answer: glucose-1-phosphate

Flashcard 5: What symptoms does essential fructosuria typically present with?_____

Answer: Asymptomatic

Flashcard 6: Essential pentosuria is a benign condition caused due to a defect in the _____ pathway.

Answer: uronic acid

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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