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CPS1

carbamoyl-phosphate synthase 1, mitochondrial

Synonymes:
CPSASE1, PHN
Espèce(s):
ID UniProtKB:
ID du gène:
  • 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
  • Zebrafish(555623) carbamoyl-phosphate synthase 1, mitochondrial
  • cow(510506) carbamoyl-phosphate synthase 1, mitochondrial
  • Horse(100050116) carbamoyl-phosphate synthase 1, mitochondrial
  • domestic cat(101099824) carbamoyl-phosphate synthase 1, mitochondrial
  • dog(488506) carbamoyl-phosphate synthase 1, mitochondrial
  • chicken(428994) carbamoyl-phosphate synthase 1, mitochondrial
  • sheep(101107584) carbamoyl-phosphate synthase 1, mitochondrial
  • domestic guinea pig(100723563) carbamoyl-phosphate synthase 1, mitochondrial
  • naked mole-rat(101696508) carbamoyl-phosphate synthase 1, mitochondrial

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Small Molecule - Allosteric Modulator

Référence du produit
Description
Espèce(s)
Carglumic Acid, United States Pharmacopeia (USP) Reference Standard,
Espèce(s)
human
N-Carbamyl-L-glutamic acid, ,
Espèce(s)
human

Antibodies

Référence du produit
Description
Espèces réactives
Application
Anti-CPS1 antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution,
Espèces réactives
human
Application
immunofluorescence
immunohistochemistry

western blot
Anti-CPS1 antibody produced in rabbit, ,
Espèces réactives
human, rat
Application
immunohistochemistry
western blot
Anti-CD8a Rabbit Monoclonal Antibody, clone RM395, affinity purified immunoglobulin,
Espèces réactives
human
Application
immunohistochemistry
western blot
Anti-CPS1 (AB2) antibody produced in rabbit, IgG fraction of antiserum,
Espèces réactives
human
Application
immunohistochemistry
western blot

esiRNA

Référence du produit
Description
Espèce(s)
MISSION® esiRNA, targeting human CPS1,
Espèce(s)
human