Carbamoyl phosphate synthetase


Carbamoyl phosphate synthetase

Carbamoyl phosphate synthetase is an enzyme that catalyzes a reaction that produces carbamoyl phosphate. This enzyme catalyzes the reaction of ATP and bicarbonate to produce carbonyl phosphate and ADP. Carbonyl phosphate reacts with ammonia to give carbamate. In turn, carbamate reacts with a second ATP to give carbamoyl phosphate plus ADP.

There are two different forms that serve very different functions:
* Carbamoyl phosphate synthetase I (mitochondria, urea cycle)
* Carbamoyl phosphate synthetase II (cytosol, pyrimidine metabolism).


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  • Carbamoyl phosphate synthetase I — Protein Name=carbamoyl phosphate synthetase 1, mitochondrial caption= Symbol=CPS1 AltSymbols= HGNCid=2323 Chromosome=2 Arm=p Band= LocusSupplementaryData= ECnumber=6.3.4.16 OMIM=608307 EntrezGene=1373 RefSeq=NM 001875 UniProt=P31327 PDB=Carbamoyl …   Wikipedia

  • carbamoyl phosphate synthetase — (CPS) car·bam·o·yl phos·phate syn·the·tase (CPS) (kahr bamґo əl fosґfāt sinґthə tās) 1. carbamoyl phosphate synthase (ammonia); called also carbamoyl phosphate synthetase I (CPSI). 2. carbamoyl phosphate synthase (glutamine… …   Medical dictionary

  • Carbamoyl phosphate synthetase I deficiency — Infobox Disease Name = PAGENAME Caption = DiseasesDB = 32671 ICD10 = ICD9 = ICD9|270.6 ICDO = OMIM = 237300 MedlinePlus = eMedicineSubj = ped eMedicineTopic = 314 MeshID = D020165 Carbamoyl phosphate synthetase I deficiency is an inherited… …   Wikipedia

  • carbamoyl phosphate synthetase deficiency — car·bam·o·yl phos·phate syn·the·tase de·fi·cien·cy (kahr bamґo əl fosґfāt sinґthə tās) an autosomal recessive aminoacidopathy caused by mutations in the CPS1 gene (locus: 2q35), which encodes carbamoyl phosphate synthase… …   Medical dictionary

  • Carbamoyl phosphate — Carbamoyl phosphate …   Wikipedia

  • Carbamoyl phosphate synthase II — protein Name=carbamoyl phosphate synthetase 2, aspartate transcarbamylase, and dihydroorotase caption= width= HGNCid=1424 Symbol=CAD AltSymbols= EntrezGene=790 OMIM=114010 RefSeq=NM 004341 UniProt=P27708 PDB= ECnumber= Chromosome=2 Arm=p Band=21… …   Wikipedia

  • carbamoyl-phosphate synthase (ammonia) — car·bam·o·yl phos·phate syn·thase (am·mo·nia) (kahr bamґo əl fosґfāt sinґthās ə moґne ə) [EC 6.3.4.16] an enzyme of the ligase class that catalyzes the synthesis of carbamoyl phosphate from ammonia and carbon dioxide, the …   Medical dictionary

  • carbamoyl phosphate — A reactive intermediate capable of transferring its carbamoyl group to an acceptor molecule, forming citrulline from ornithine in the urea cycle, and ureidosuccinic acid from aspartic acid in …   Medical dictionary

  • Carbamoylphosphat-Synthetase I — Masse/Länge Primärstruktur 1462 Aminosäuren …   Deutsch Wikipedia

  • Glutathione synthetase deficiency — Classification and external resources Glutathione OMIM 266130 …   Wikipedia


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