CPS1
carbamoyl-phosphate synthase 1, mitochondrial
CPSASE1, PHN
Human CPS1 (1373), Mouse Cps1 (227231), Rat Cps1 (497840), Horse CPS1 (100050116), sheep CPS1 (101107584), Zebrafish cps1 (555623), naked mole-rat Cps1 (101696508), chicken CPS1 (428994), domestic guinea pig Cps1 (100723563), dog CPS1 (488506) ...More
- Human(1373) Summary: The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This reaction is the first committed step of the urea cycle, which is important in the removal of excess urea from cells. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.[provided by RefSeq, May 2010]
- Mouse(227231) carbamoyl-phosphate synthetase 1
- Rat(497840) carbamoyl-phosphate synthetase 1
- Horse(100050116) carbamoyl-phosphate synthase 1, mitochondrial
- sheep(101107584) carbamoyl-phosphate synthase 1, mitochondrial
- Zebrafish(555623) carbamoyl-phosphate synthase 1, mitochondrial
- naked mole-rat(101696508) carbamoyl-phosphate synthase 1, mitochondrial
- chicken(428994) carbamoyl-phosphate synthase 1, mitochondrial
- domestic guinea pig(100723563) carbamoyl-phosphate synthase 1, mitochondrial
- dog(488506) carbamoyl-phosphate synthase 1, mitochondrial
- domestic cat(101099824) carbamoyl-phosphate synthase 1, mitochondrial
- cow(510506) carbamoyl-phosphate synthase 1, mitochondrial
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Antibodies
Anti-CPS1 (AB2) antibody produced in rabbit, IgG fraction of antiserum,
human
immunohistochemistry
western blot
western blot
Anti-CPS1 antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution,
human
immunofluorescence
immunohistochemistry
western blot
immunohistochemistry
western blot