Urea cycle disorders — Flashcards

Flashcard 1: Hyperammonemia results in excess NH3, which depletes _____, leading to inhibition of the TCA cycle

Answer: α-ketoglutarate

Flashcard 2: Ornithine transcarbamylase (OTC) deficiency presents with _____ BUN

Answer: decreased

Flashcard 3: Ornithine transcarbamylase (OTC) deficiency presents with _____ serum glutamine

Answer: increased

Flashcard 4: Does ornithine transcarbamylase deficiency present with orotic aciduria? _____

Answer: Yes

Flashcard 5: Part of the treatment for hyperammonemia is limiting _____ in the diet

Answer: protein

Flashcard 6: What is the most common urea cycle disorder? _____

Answer: Ornithine transcarbamylase (OTC) deficiency

Flashcard 7: Disorders of the urea cycle interfere with the body's ability to eliminate _____

Answer: ammonia (NH3)

Flashcard 8: What stainable protein is abundant in astrocytes?

Answer: GFAP

Flashcard 9: What enzyme converts pyruvate to alanine?

Answer: alanine aminotransferase (ALT)

Flashcard 10: Role of myeloperoxidase in neutrophils:

Answer: Plays a role in respiratory burst (catalyzes formation of HOCl from H2O2)

Extra: Myeloperoxidase (MPO) is found in the azurophilic granules of neutrophils (PMNs). During the respiratory burst, it converts Hydrogen Peroxide (H2O2) and Chloride ions into Hypochlorous acid (HOCl - bleach), which is a potent bactericidal agent.