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Uroporphyrinogen III Synthetase

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Definition: An enzyme that catalyzes the cyclization of hydroxymethylbilane to yield UROPORPHYRINOGEN III and water. It is the fourth enzyme in the 8-enzyme biosynthetic pathway of HEME, and is encoded by UROS gene. Mutations of UROS gene result in CONGENITAL ERYTHROPOIETIC PORPHYRIA.     
See Also Porphyria, Erythropoietic 		Other names Uroporphyrinogen-III Synthase; Uroporphyrinogen Isomerase; Uroporphyrinogen III Cosynthetase; Cosynthase; Uroporphyrinogen III Synthase; Synthetase, Uroporphyrinogen III; Synthase, Uroporphyrinogen-III; Isomerase, Uroporphyrinogen; Cosynthetase, Uroporphyrinogen III
 
SubstanceCAS Registry & nameCategoriesSourceDrugs*
19-methyl-1-hydroxymethylbilane  0   *Uroporphyrinogens Uroporphyrinogen III Synthetase/antagonists & inhibitors. Bioorg Med Chem 1994 Apr;2(4):267-77
19-bromo-1-hydroxymethylbilane  0   *Uroporphyrinogens Uroporphyrinogen III Synthetase/antagonists & inhibitors. Bioorg Med Chem 1994 Apr;2(4):267-77

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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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