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Hydroxymethylbilane Synthase

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Definition: An enzyme that catalyzes the tetrapolymerization of the monopyrrole PORPHOBILINOGEN into the hydroxymethylbilane preuroporphyrinogen (UROPORPHYRINOGENS) in several discrete steps. It is the third enzyme in the 8-enzyme biosynthetic pathway of HEME. In humans, deficiency in this enzyme encoded by HMBS (or PBGD) gene results in a form of neurological porphyria (PORPHYRIA, ACUTE INTERMITTENT). This enzyme was formerly listed as EC 4.3.1.8     
See Also Porphyrias 		Other names Uroporphyrinogen I Synthase; Porphobilinogen Deaminase; Porphobilinogen Ammonia-Lyase; Synthetase, Preuroporphyrinogen; Synthetase, Pre-uroporphyrinogen; Synthetase, Hydroxymethylbilane; Synthase, Uroporphyrinogen I; Synthase, Hydroxymethylbilane; Pre uroporphyrinogen Synthetase; Porphobilinogen Ammonia Lyase; Deaminase, Porphobilinogen; Ammonia-Lyase, Porphobilinogen; Preuroporphyrinogen Synthetase; Pre-uroporphyrinogen Synthetase; Hydroxymethylbilane Synthetase
 
SubstanceCAS Registry & nameCategoriesSourceDrugs*
hemC protein, E coli  EC 4.3.1.8   *Hydroxymethylbilane Synthase *Escherichia coli Proteins. Arch Microbiol 1991;156(4):281-9
hydroxymethylbilane  71861-60-4 21H-biline-2,7,12,17-tetrapropanoic acid, 3,8,13,18-tetrakis(carboxymethyl)-5,10,15,22,23,24-hexahydro-19-(hydroxymethyl)  *Uroporphyrinogens Hydroxymethylbilane Synthase. Nature 1980;285(5759):19

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Sources: NLM Medical Subject Headings, NIH UMLS, Drugs@FDA, FDA AERS original data copyright United States Government. No endorsement implied. Last modified 6/6/2012
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