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hydroxymetylbilánsyntáza

  1. Subject h.hydroxymetylbilánsyntáza
    Subject h.Hydroxymethylbilane Synthase
    Entry termsporfobilinogéndeamináza
    English X referencesPorphobilinogen Ammonia-Lyase
    Porphobilinogen Deaminase
    Uroporphyrinogen I Synthase
    Scope note in EnglishAn 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
    See also reference (FX) in Slovak porfýrie
    See also reference (FX) in English Porphyrias
    Links (2) - MeSH descriptor
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Number of the records: 1  

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