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Hydroksimetyylibilaanisyntaasi
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Anmärkning: | 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
X |
historyNote*: | 98(75)
X |
publicMeSHNote*: | 98; see PORPHOBILINOGEN DEAMINASE 1991-97, see AMMONIA-LYASES 1975-90
X |
activeMeSHYear*: | |
dateCreated*: | 1974-11-19X |
dateEstablished*: | 1991-01-01X |
dateRevised*: | 2005-06-30X |
recordAuthorizer*: | sjnX |
recordMaintainer*: | jmpX |
recordOriginator*: | NLMX |
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Termer och motsvarande begrepp: | Hydroxymethylbilane Synthase (en) XPreuroporphyrinogen Synthetase (en, ersatt) Uroporphyrinogen I Synthase (en, ersatt) Pre-uroporphyrinogen Synthetase (en, ersatt) Porphobilinogen Deaminase (en, ersatt) Porphobilinogen Ammonia-Lyase (en, ersatt) Hydroxymethylbilane Synthetase (en, ersatt) |
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