Carbamoyl phosphate synthetase I

Carbamoyl phosphate synthetase I ( CPS1 ) is the enzyme that catalyzes the addition of ammonia, carbon dioxide and ATP carbamoyl. This reaction takes place in the mitochondria of vertebrates and is the rate-limiting step of the urea cycle. In humans is CPS1 mainly active in the liver and in the small intestine. Mutations in the gene CPS1 and this can lead to CPS deficiency and hyperammonemia pulmonary hypertension of the newborn. A variant is associated with risk for neonatal arginine deficiency and necrotizing enterocolitis.

The CPS1 transcription is controlled both by cAMP, and glucocorticoid signaling pathways. The expression of CPS1 is suppressed in the three human liver cancer cell lines. CPS1 is identical to the antigen used in surgery Hep par 1 antibody.

Catalyzed reaction

Which is made in three steps reaction starts by a total of two molecules of ATP and one molecule each of ammonia and carbon dioxide to two molecules of ADP and one molecule of carbamoyl phosphate and phosphate.

Protein isoform Human Genome Organisation Ligase Addition reaction Adenosine triphosphate Digital Object Identifier
163194
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