Disorders of branched-chain amino acids (MSUD) — Flashcards

Flashcard 1: Which amino acids (3) are characterized by branched side chains?_____

Answer: Valine, Leucine, Isoleucine

Flashcard 2: Dietary ingestion of which amino acids (3) should be restricted in patients with maple syrup urine disease? _____

Answer: Leucine, Isoleucine, Valine

Flashcard 3: _____ disease presents with vomiting, poor feeding, and urine that smells like maple syrup/burnt sugar

Answer: Maple syrup urine

Flashcard 4: What stainable protein is abundant in astrocytes?

Answer: GFAP

Flashcard 5: What enzyme converts pyruvate to alanine?

Answer: alanine aminotransferase (ALT)

Flashcard 6: myeloperoxidase

Answer: respiratory burst

Extra: H2O2HOClPMNs

Flashcard 7: superoxide dismutase

Answer: respiratory burst

Extra: O2-H2O2

Flashcard 8: The Golgi makes modifications to some amino acids trafficking through it. What are the modifications made to threonine?

Answer: O-oligosaccharide

Flashcard 9: The Golgi makes modifications to some amino acids trafficking through it. What are the modifications made to asparagine?

Answer: N-oligosaccharide

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

Answer: O-oligosaccharide