Fumarate produced by cleavage of argininosuccinate enters the citric acid cycle, which along with this cycle forms the Krebs bi-cycle.
Deficiency of an intermediate in this process resulting from an autosomal recessive mutation can cause spasticity and ataxia. That enzymatic intermediate competes with nitric oxide synthase to control sexual behavior, and inhibition of that intermediate by ABH restores natural sexual function. That intermediate in this process also produces an amino acid derivative that is the starting point for polyamine synthesis and reacts with carbamoyl phosphate synthetase to form citrulline. Also in this process, an aspartate generated by transamination in mitochondria is condensed with a citrullyl-AMP intermediate to produce argininosuccinate, the precursor to the two products of this process. Fumarate produced by cleavage of argininosuccinate enters the citric acid cycle, which along with this cycle forms the Krebs bi-cycle. For 10 points, name this process occurring in the human liver and kidney which converts ammonia to the namesake amide.
Deficiency of an intermediate in this process resulting from an autosomal recessive mutation can cause spasticity and ataxia.
urea cycle