urea cycle

L-Ornithine hydrochloride ((S)-2,5-Diaminopentanoic acid) has an antifatigue effect by increasing the efficiency of energy consumption and promoting the excretion of ammonia. It is one of the key reactants in the urea cycle.

2-Amino-5-ureidopentanoic acid (Citrulline) exists in the liver, is not a protein component, is an important intermediate in the human urea cycle, can be combined with ornithine, arginine to treat hyperammonemia.

4-Acetamidobutanoic acid (4-ACETAMIDOBUTYRIC ACID) can be found in blood, feces, and urine, as well as in human prostate tissue. 4-Acetamidobutanoic acid exists in all eukaryotes, ranging from yeast to humans. 4-Acetamidobutanoic acid is a GABA derivative, a product of the urea cycle and the metabolism of amino groups, and the product of NAD-linked aldehyde dehydrogenase (EC 1.2.1.3) (KEGG).

N-Acetyl-L-glutamic acid (Ac-L-Glu-OH) (NAcGlu) is an acetylated amino acid. NAcGlu is biosynthesized from glutamic acid and acetyl-CoA by the enzyme N-acetyl glutamate synthase (NAGS). NAcGlu activates carbamoyl phosphate synthetase in the urea cycle. A deficiency in N-acetyl glutamate synthase or a genetic mutation in the gene coding for the enzyme will lead to urea cycle failure in which ammonia is not converted to urea, but rather accumulated in the blood leading to the condition called Type I hyperammonemia.

L-Homocitrulline (Homocitrulline) is a metabolite that can be detected in larger amounts in the urine of individuals with urea cycle disorders. The accumulation of carbamyl phosphate due to a depleted supply of ornithine for the urea cycle may be responsible for the enhanced synthesis of L-Homocitrulline and homoarginine in some cases.