UROPORFIRINOGEN I SINTAZA
(engl. uroporphyrinogen I synthase), enzim iz roda liaza (EC 4.3.1.8) koji katalizuje reakciju 4- –
porfobilinogen = uroporfirinogen I + 4 NH3. Usled deficita ovog enzima javlja se akutna
intermitentna porfirija, koja se nasleđuje na autosomno dominantan način. Ovaj enzim se naziva i
porfobilinogen deaminaza.
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