holocarboxylase synthetase

holocarboxylase synthetase
One of several enzymes that biotinylate other proteins ( e.g., carboxylases); a deficiency of h. will result in organic acidemia.

* * *

holo·car·boxy·lase syn·the·tase (hōl″o-kahr-bokґsə-lās sinґthə-tās) an enzyme catalyzing the ATP-dependent biotinylation of the apoenzyme forms of the carboxylases acetyl-CoA carboxylase, methylcrotonoyl-CoA carboxylase, propionyl-CoA carboxylase, and pyruvate carboxylase, forming the active holoenzymes.

Medical dictionary. 2011.

Игры ⚽ Нужно сделать НИР?

Look at other dictionaries:

  • Holocarboxylase synthetase — protein Name=holocarboxylase synthetase (biotin (proprionyl Coenzyme A carboxylase (ATP hydrolysing)) ligase) caption= width= HGNCid=4976 Symbol=HLCS AltSymbols= EntrezGene=3141 OMIM=609018 RefSeq=NM 000411 UniProt=P50747 PDB= ECnumber=6.3.4.10… …   Wikipedia

  • Holocarboxylase synthetase deficiency — Infobox Disease Name = PAGENAME Caption = Biotin DiseasesDB = 32709 ICD10 = ICD9 = ICDO = OMIM = 253270 MedlinePlus = eMedicineSubj = ped eMedicineTopic = 1020 MeshID = D028922 | Holocarboxylase synthetase deficiency is an inherited metabolic… …   Wikipedia

  • holocarboxylase synthetase deficiency — holo·car·boxy·lase syn·the·tase de·fi·cien·cy (hōl″o kahr bokґsə lās sinґthə tās) a biotin responsive, autosomal recessive aminoacidopathy caused by mutations in the HLCS gene (locus: 21q22.1), which encodes… …   Medical dictionary

  • Holocarboxilasa sintetasa — holocarboxylase synthetase (biotin (proprionyl Coenzyme A carboxylase (ATP hydrolysing)) ligase) HUGO 4976 Símbolo HLCS Datos genéticos …   Wikipedia Español

  • Biotin — Biotin[1] …   Wikipedia

  • Multiple carboxylase deficiency — Classification and external resources MeSH D009100 Multiple carboxylase deficiency is a form of metabolic disorder involving failures of carboxylation enzymes. The deficiency can be in biotinidase or holocarboxylase synth …   Wikipedia

  • Biotin — Strukturformel Allgemeines Trivialname Vitamin B7 Vitamin B8 Vitamin H …   Deutsch Wikipedia

  • Biotin-(acetyl-CoA-carboxylase) ligase — In enzymology, a biotin [acetyl CoA carboxylase] ligase (EC number|6.3.4.15) is an enzyme that catalyzes the chemical reaction:ATP + biotin + apo [acetyl CoA:carbon dioxide ligase (ADP forming)] ightleftharpoons AMP + diphosphate + [acetyl… …   Wikipedia

  • Biotin-(methylcrotonoyl-CoA-carboxylase) ligase — In enzymology, a biotin [methylcrotonoyl CoA carboxylase] ligase (EC number|6.3.4.11) is an enzyme that catalyzes the chemical reaction:ATP + biotin + apo [3 methylcrotonoyl CoA:carbon dioxide ligase (ADP forming)] ightleftharpoons AMP +… …   Wikipedia

  • CTP synthase — Cytidine triphosphate Identifiers EC number 6.3.4.2 …   Wikipedia

Share the article and excerpts

Direct link
Do a right-click on the link above
and select “Copy Link”