Hydroxymethylbilane Synthase

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

Year introduced: 1998(1975)

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Tree Number(s): D08.811.913.225.575

MeSH Unique ID: D011163

Registry Number: EC 2.5.1.61

Entry Terms:

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

Previous Indexing:

• Ammonia-Lyases (1973-1974)
• Porphobilinogen (1973-1974)

See Also:

• Porphyrias

All MeSH Categories
Chemicals and Drugs Category
Enzymes and Coenzymes
Enzymes
Transferases
Alkyl and Aryl Transferases
Hydroxymethylbilane Synthase