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CPS1

carbamoyl-phosphate synthase 1, mitochondrial

Synonyms:
CPSASE1, PHN
Species:
UniProtKB ID:
Gene ID:
  • 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

Custom & Knockdown Gene Products

  • siRNA

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  • Custom Gene Products

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Small Molecule - Allosteric Modulator

Product Number
Description
Species
N-Carbamyl-L-glutamic acid, ,
Species
human
Carglumic Acid, United States Pharmacopeia (USP) Reference Standard,
Species
human

Antibodies

Product Number
Description
Species Reactivity
Application
Anti-CPS1 (AB2) antibody produced in rabbit, IgG fraction of antiserum,
Species Reactivity
human
Application
immunohistochemistry
western blot
Anti-CPS1 antibody produced in rabbit, ,
Species Reactivity
human, rat
Application
immunohistochemistry
western blot
Anti-CPS1 antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution,
Species Reactivity
human
Application
immunofluorescence
immunohistochemistry

western blot
Anti-CD8a Rabbit Monoclonal Antibody, clone RM395, affinity purified immunoglobulin,
Species Reactivity
human
Application
immunohistochemistry
western blot

esiRNA

Product Number
Description
Species
MISSION® esiRNA, targeting human CPS1,
Species
human