hydroxymethylbilane synthase

hydroxymethylbilane synthase
hy·droxy·meth·yl·bil·ane syn·thase (hi-drok″se-meth″əl-bilґān sinґthās) [EC 2.5.1.61] an enzyme of the transferase class that catalyzes the deamination and condensation of four molecules of porphobilinogen to form a linear tetrapyrrole intermediate, hydroxymethylbilane, which cyclizes to form uroporphyrinogen. Alone, the enzyme produces uroporphyrinogen I; in the presence of uroporphyrinogen synthase it produces uroporphyrinogen III, the physiologically important isomer. Deficiency of the enzyme, an autosomal dominant trait, results in acute intermittent porphyria. Called also porphobilinogen deaminase and uroporphyrinogen I synthase.

Medical dictionary. 2011.

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  • Hydroxymethylbilane — Chembox new ImageFile=Hydroxymethylbilane.svg ImageSize= IUPACName= OtherNames= Section1= Chembox Identifiers CASNo=73023 76 4 PubChem=788 ChemSpiderID = 767 SMILES= MeSHName=hydroxymethylbilane Section2= Chembox Properties Formula=C40H46N4O17… …   Wikipedia

  • uroporphyrinogen I synthase — uro·por·phy·rin·o·gen I syn·thase (u″ro por″fə rinґə jən sinґthās) hydroxymethylbilane synthase …   Medical dictionary

  • uroporphyrinogen-III synthase — uro·por·phy·rin·o·gen III syn·thase (u″ro por″fə rinґə jən sinґthās) [EC 4.2.1.75] an enzyme of the lyase class that acts concertedly with hydroxymethylbilane synthase to convert porphobilinogen to uroporphyrinogen …   Medical dictionary

  • Porphobilinogen deaminase — (or hydroxymethylbilane synthase) is an enzyme involved in the third step of the metabolism of porphyrin, converting porphobilinogen into hydroxymethyl bilane. The enzyme has the unique cofactor dipyrromethane. Defective activity of this enzyme… …   Wikipedia

  • List of EC numbers (EC 2) — This list contains a list of EC numbers for the second group, EC 2, transferases, placed in numerical order as determined by the Nomenclature Committee of the International Union of Biochemistry and Molecular Biology.EC 2.1: Transferring One… …   Wikipedia

  • Heme — A heme (American English) or haem (British English) is a prosthetic group that consists of an iron atom contained in the center of a large heterocyclic organic ring called a porphyrin . Not all porphyrins contain iron, but a substantial fraction… …   Wikipedia

  • PPOX — Protoporphyrinogen oxidase, also known as PPOX, is a human gene.cite web | title = Entrez Gene: PPOX protoporphyrinogen oxidase| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene Cmd=ShowDetailView TermToSearch=5498| accessdate = ] PBB… …   Wikipedia

  • porphobilinogen deaminase — por·pho·bi·lin·o·gen de·am·in·ase (por″fo bĭ linґə jən de amґin ās) hydroxymethylbilane synthase …   Medical dictionary

  • acute intermittent porphyria — (AIP) an autosomal dominant hepatic porphyria caused by mutation in the HMBS gene (locus: 11q23.3), which encodes hydroxymethylbilane synthase; it is manifested by recurrent attacks of abdominal pain, gastrointestinal dysfunction, and neurologic… …   Medical dictionary

  • Porphyrie — Classification internationale des maladies CIM 10 : E80.0 → E80.2 La porphyrie est une affection caractérisée par la présence, dans l organisme, de quantités massives de porphyrines, molécules précurseurs de l hème (partie non protéique de l… …   Wikipédia en Français

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