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Uroporphyrinogen Decarboxylase MeSH Descriptor Data 2025


MeSH Heading
Uroporphyrinogen Decarboxylase
Tree Number(s)
D08.811.520.224.125.900
Unique ID
D014575
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D014575
Annotation
/defic permitted but consider also PORPHYRIA CUTANEA TARDA and HEPATOERYTHROPOIETIC PORPHYRIA
Scope Note
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.
Entry Term(s)
Uroporphyrinogen Carboxy-Lyase
Uroporphyrinogen III Decarboxylase
Registry Numbers
EC 4.1.1.37
Related Numbers
9024-70-8
CAS Type 1 Name
Uroporphyrinogen-III carboxy-lyase
Previous Indexing
Carboxy-Lyases (1974-1975)
Porphyrinogens (1975)
Porphyrins (1974)
Public MeSH Note
91; was see under CARBOXY-LYASES 1976-90
History Note
91(76); was see under CARBOXY-LYASES 1976-90
Date Established
1991/01/01
Date of Entry
1975/07/25
Revision Date
2013/07/09
Uroporphyrinogen Decarboxylase Preferred
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