Branched-chain amino acid metabolism — Flashcards

Flashcard 1: What is the mode of inheritance of maple syrup urine disease?_____

Answer: Autosomal recessive

Flashcard 2: Maple syrup urine disease is characterized by blocked degradation of _____ amino acids

Answer: branched

Flashcard 3: Are the essential amino acids methionine, valine, and histidine ketogenic or glucogenic? _____

Answer: Glucogenic

Flashcard 4: Thiamine pyrophosphate (B1) is a cofactor for the enzyme _____ (glycolysis)

Answer: pyruvate dehydrogenase

Flashcard 5: Treatment of maple syrup urine disease includes high-dose _____ supplementation

Answer: thiamine

Flashcard 6: What stainable protein is abundant in astrocytes?

Answer: GFAP

Flashcard 7: What enzyme converts pyruvate to alanine?

Answer: alanine aminotransferase (ALT)

Flashcard 8: myeloperoxidase

Answer: respiratory burst

Extra: H2O2HOClPMNs

Flashcard 9: superoxide dismutase

Answer: respiratory burst

Extra: O2-H2O2

Flashcard 10: The Golgi makes modifications to some amino acids trafficking through it. What are the modifications made to threonine?

Answer: O-oligosaccharide