A mutation in the SLC25A15 gene leads to a deficiency in a translocase enzyme integral to this pathway and is known as HHH syndrome.
A mutation in the SLC25A15 gene leads to a deficiency in a translocase enzyme integral to this pathway and is known as HHH syndrome. One reaction in this pathway involves the transamination of oxaloacetate into aspartate, while other steps in this pathway result in the production of 2 NADH molecules. ARG1 catalyzes the final step of this pathway by hydrolyzing cytoplasmic L-arginine. N-acetyl glutamate allosterically regulates the main enzyme of this pathway, carbamoyl phosphatase synthetase, which generates its nitrogenous product from the hydrolysis of three ATP to two ADP and one AMP. Also known as the ornithine cycle, for 10 points, give the name of this pathway that takes place primarily in the liver and produces its namesake waste product containing two amine groups bonded to a carbonyl group.
A mutation in the SLC25A15 gene leads to a deficiency in a translocase enzyme integral to this pathway and is known as HHH syndrome.
urea cycle [accept "ornithine cycle" before mention]