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Hydroxymethylbilane Synthase
Hierarchy: | ∟ ∟ ∟ ∟ ∟ Hydroxymethylbilane Synthase 14 coordinate concepts1 subordinate concept∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ ∟ |
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historyNote*: | 98(75)
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publicMeSHNote*: | 98; see PORPHOBILINOGEN DEAMINASE 1991-97, see AMMONIA-LYASES 1975-90
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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
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activeMeSHYear*: | |
dateCreated*: | 1974-11-19X |
dateEstablished*: | 1991-01-01X |
dateRevised*: | 2005-06-30X |
recordAuthorizer*: | sjnX |
recordMaintainer*: | jmpX |
recordOriginator*: | NLMX |
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Labels and equivalent concepts: | Hydroksimetyylibilaanisyntaasi (fi) XUroporphyrinogen I Synthase (en, replaced) Preuroporphyrinogen Synthetase (en, replaced) Pre-uroporphyrinogen Synthetase (en, replaced) Hydroxymethylbilane Synthetase (en, replaced) Porphobilinogen Ammonia-Lyase (en, replaced) Porphobilinogen Deaminase (en, replaced) Sintasa Hidroximetilbilano (es) συνθάση ουροπορφυρινογόνου Ι (el) Hydroxymethylbilan-Synthase (de) hydroxymethylbilansynthasa (cs) Porfobilinógeno Amoniacoliasa (es, replaced) Porfobilinógeno Desaminasa (es, replaced) Uroporfirinógeno I Sintasa (es, replaced) Porphobilinogen-Desaminase (de, replaced) Porphobilinogen-Ammoniak-Lyase (de, replaced) |
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