Uroporphyrinogen Decarboxylase

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.

Year introduced: 1991(1976)

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Tree Number(s): D08.811.520.224.125.900

MeSH Unique ID: D014575

Registry Number: EC 4.1.1.37

Entry Terms:

• Decarboxylase, Uroporphyrinogen
• Uroporphyrinogen Carboxy-Lyase
• Carboxy-Lyase, Uroporphyrinogen
• Uroporphyrinogen Carboxy Lyase
• Uroporphyrinogen III Decarboxylase
• Decarboxylase, Uroporphyrinogen III

Previous Indexing:

• Carboxy-Lyases (1974-1975)
• Porphyrinogens (1975)
• Porphyrins (1974)

All MeSH Categories
Chemicals and Drugs Category
Enzymes and Coenzymes
Enzymes
Lyases
Carbon-Carbon Lyases
Carboxy-Lyases
Uroporphyrinogen Decarboxylase