" /> uroporphyrinogen decarboxylase - CISMeF





Preferred Label : uroporphyrinogen decarboxylase;

MeSH definition : An enzyme that catalyzes the decarboxylation of UROPORPHYRINOGEN III to coproporphyrinogen III by the conversion of four acetate groups to four methyl groups. It is the fifth enzyme in the 8-enzyme biosynthetic pathway of HEME. Several forms of cutaneous PORPHYRIAS are results of this enzyme deficiency as in PORPHYRIA CUTANEA TARDA; and HEPATOERYTHROPOIETIC PORPHYRIA.;

MeSH synonym : carboxy-lyase, uroporphyrinogen; decarboxylase, uroporphyrinogen; decarboxylase, uroporphyrinogen iii; uroporphyrinogen carboxy lyase; uroporphyrinogen carboxy-lyase; uroporphyrinogen iii decarboxylase;

MeSH CAS label : Uroporphyrinogen-III carboxy-lyase;

MeSH Related Number : 9024-70-8 (CAS RN);

Registry Number MeSH : EC 4.1.1.37;

MeSH annotation : /defic permitted but consider also PORPHYRIA CUTANEA TARDA and HEPATOERYTHROPOIETIC PORPHYRIA;

Is substance : O;

UNII : EC 4.1.1.37;

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An enzyme that catalyzes the decarboxylation of UROPORPHYRINOGEN III to coproporphyrinogen III by the conversion of four acetate groups to four methyl groups. It is the fifth enzyme in the 8-enzyme biosynthetic pathway of HEME. Several forms of cutaneous PORPHYRIAS are results of this enzyme deficiency as in PORPHYRIA CUTANEA TARDA; and HEPATOERYTHROPOIETIC PORPHYRIA.

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10/05/2024


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