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Enzymes and Coenzymes > Enzymes > Transferases > Alkyl and Aryl Transferases > Hydroxymethylbilane Synthase

Preferred term

Hydroxymethylbilane Synthase  

Type

  • Topical Descriptor

Broader concept

Related concepts

Entry terms

  • Ammonia-Lyase, Porphobilinogen
  • Deaminase, Porphobilinogen
  • Hydroxymethylbilane Synthetase
  • Porphobilinogen Ammonia Lyase
  • Porphobilinogen Ammonia-Lyase
  • Porphobilinogen Deaminase
  • Pre uroporphyrinogen Synthetase
  • Pre-uroporphyrinogen Synthetase
  • Preuroporphyrinogen Synthetase
  • Synthase, Uroporphyrinogen I
  • Synthetase, Hydroxymethylbilane
  • Synthetase, Pre-uroporphyrinogen
  • Synthetase, Preuroporphyrinogen
  • Uroporphyrinogen I Synthase

Scope note

  • 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

History note

  • 98(75)

In other languages

URI

http://www.yso.fi/onto/mesh/D011163

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