Urea Cycle — Flashcards

Flashcard 1: How does hyperammonemia deplete alpha-ketoglutarate?

Answer: 1. The excess free ammonia binds to glutamate and forms glutamine. 2. To compensate for the reduced glutamate, alpha-ketoglutarate is aminated to glutamate.

Flashcard 2: Carbamoyl phosphate synthetase I deficiency presents with _____ NH4+ and hyper-ammonemia

Answer: increased

Flashcard 3: NH3 is excreted in the urine primarily in the form of _____ (from the liver) and NH4+ (from glutamine breakdown)

Answer: urea

Flashcard 4: Arginine may be broken down into _____ and urea via the enzyme arginase

Answer: ornithine

Flashcard 5: Hyperammonemia type 1 occurs due to deficiency of the enzyme _____

Answer: CPS-1

Flashcard 6: The urea cycle begins with _____, CO2, and 2 ATP

Answer: NH4+

Flashcard 7: Ornithine transcarbamylase (OTC) deficiency presents with hyperammonemia and increased _____.

Answer: orotic acid

Flashcard 8: pulses are deficient in _____ amino acids

Answer: cysteine and methionine

Extra: 

Flashcard 9: Cereals are deficient in _____ amino acids

Answer: threonine and lysine

Flashcard 10: Taurine is essential for _____ and retinal development in preterm infants.

Answer: axonal growth