CPS1 Recombinant Rabbit Monoclonal Antibody [JB40-33] (ET7107-69)

UNIPROT #

P31327

PROTEIN NAME

CPS1

SYNONYMS

Carbamoyl phosphate synthase [ammonia] antibody;Carbamoyl phosphate synthase [ammonia] mitochondrial antibody;Carbamoyl phosphate synthase antibody;Carbamoyl phosphate synthetase 1 antibody;Carbamoyl phosphate synthetase 1 mitochondrial antibody;Carbamoyl phosphate synthetase I antibody;Carbamoyl-phosphate synthase [ammonia] antibody;Carbamoyl-phosphate synthetase I antibody;Carbamoylphosphate synthase antibody;Carbamoylphosphate synthetase 1 antibody;Carbamoylphosphate synthetase I antibody;CPS 1 antibody;Cps1 antibody;CPSase 1 antibody;CPSase I antibody;CPSASE1 antibody;CPSM_HUMAN antibody;mitochondrial antibody;MS738 antibody

TISSUE SPECIFICITY

Primarily in the liver and small intestine.

POST-TRANSLATIONAL MODIFICATION

Undergoes proteolytic cleavage in the C-terminal region corresponding to the loss of approximately 12 AA residues from the C-terminus.; Succinylated at Lys-287 and Lys-1291. Desuccinylated at Lys-1291 by SIRT5, leading to activation (By similarity).; Glutarylated. Glutarylation levels increase during fasting. Deglutarylated by SIRT5 at Lys-55, Lys-219, Lys-412, Lys-889, Lys-892, Lys-915, Lys-1360 and Lys-1486, leading to activation.

SUBCELLULAR LOCATION

Mitochondrion, Nucleus.

FUNCTION

The multicomplex protein, carbamoyl-phosphate synthetase-aspartate carbamoyl transferase-dihydro-orotase (CAD), consists of three distinct proteins, carbamoyl phosphate synthetase 2 (CPS2), aspartate transcarbamylase, and dihydro-orotase, which catalyze the second and third steps of pyrimidine biosynthesis. CAD is allosterically regulated by the phosphorylation of CPS2 by cyclic AMP-dependent protein kinase, and this activation enables CPS2 to catalyze the rate-limiting step of pyrimidine synthesis. CAD is expressed in brain and skeletal muscle. A related protein, carbamoyl phosphate synthetase 1 (CPS1) is expressed in liver. CPS1 catalyzes the rate-limiting step in the urea cycle, and deficiency of CPS1 is an autosomal recessive disorder that causes hyperammonemia.