pyruvate carboxylase

pyruvate carboxylase
pyruvate carboxylase n an enzyme that contains biotin as a prosthetic group and in the presence of acetyl coenzyme A catalyzes the fixation of carbon dioxide by pyruvate to form oxalacetate

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py·ru·vate car·box·y·lase (piґroo-vāt kahr-bokґsə-lās) [EC 6.4.1.1] an enzyme of the ligase class that catalyzes the irreversible carboxylation of pyruvate to form oxaloacetate. The enzyme is a mitochondrial protein containing a biotin prosthetic group, requiring Mg2+ or Mn2+ and acetyl CoA, and occurs in liver but not in muscle. The reaction is necessary for gluconeogenesis from lactate or amino acids forming pyruvate and also provides four-carbon compounds for the tricarboxylic acid cycle. Deficiency of the enzyme, an autosomal recessive trait, causes severe psychomotor retardation and lactic acidosis in infants; there is a particularly severe, rapidly fatal form, in which hyperammonemia, hypercitrullinemia, and hyperlysinemia are also present.

Medical dictionary. 2011.

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  • Pyruvate carboxylase — Protein Name=PAGENAME caption= Symbol=PC AltSymbols= HGNCid=8636 Chromosome=11 Arm=q Band=11 LocusSupplementaryData= q13.1 ECnumber=6.4.1.1 OMIM=608786 EntrezGene=5091 RefSeq=NM 000920 UniProt=P11498 PDB=Pyruvate carboxylase is an enzyme of the… …   Wikipedia

  • Pyruvate carboxylase deficiency — Classification and external resources Oxaloacetate is the product of pyruvate carboxylase ICD 10 E …   Wikipedia

  • pyruvate carboxylase — An enzyme that catalyses the formation of oxaloacetate from pyruvate, CO2 and ATP in gluconeogenesis …   Dictionary of molecular biology

  • Pyruvate dehydrogenase complex — (PDC) is a complex of three enzymes that transform pyruvate into acetyl CoA by a process called pyruvate decarboxylation. Acetyl CoA may then be used in the citric acid cycle to carry out cellular respiration, and this complex links the… …   Wikipedia

  • Pyruvate kinase — is an enzyme involved in glycolysis. It catalyzes the transfer of a phosphate group from phosphoenolpyruvate (PEP) to ADP, yielding one molecule of pyruvate and one molecule of ATP. ReactionThe reaction with pyruvate kinase: pyruvate kinase PEP > …   Wikipedia

  • Carboxylase — La carboxylase est une enzyme qui assure l incorporation du CO2 dans les molécules intermédiaires pour la synthèse de glucides. Plus précisément il s agit de la pyruvate carboxylase. Cette enzyme va transformer un pyruvate, en lui ajoutant du CO2 …   Wikipédia en Français

  • Pyruvate — Acide pyruvique Acide pyruvique acide pyruvique Général …   Wikipédia en Français

  • pyruvate — A salt or ester of pyruvic acid. active p. an intermediate formed in the oxidative decarboxylation of p.. Cf.:p. dehydrogenase (lipoamide). SYN: α lactyl thiamin pyrophosphate. p. carboxylase ligase …   Medical dictionary

  • carboxylase — 1. One of several carboxy lyases, trivially named carboxylases or decarboxylases (EC 4.1.1.x), catalyzing the addition of CO2 to all or part of another molecule to create an additional COOH group ( e.g., ribulose 1,5 bisphosphate c.). 2. Obsolete …   Medical dictionary

  • Propionyl-CoA carboxylase — PCCA redirects here. For other uses, see PCCA (disambiguation). Propionyl CoA carboxylase Identifiers EC number 6.4.1.3 CAS number …   Wikipedia

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