- Concept UI
- M0022406
- Registry Numbers
- EC 4.1.1.37
- Related Numbers
- 9024-70-8
- 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.
- Terms
-
Uroporphyrinogen Decarboxylase
Preferred Term
Term UI
T042641
Date01/01/1999
LexicalTag
NON
ThesaurusID
NLM (1976)
-
Uroporphyrinogen Carboxy-Lyase
Term UI
T042640
Date07/25/1975
LexicalTag
NON
ThesaurusID
UNK (19XX)
-
Uroporphyrinogen III Decarboxylase
Term UI
T551700
Date09/26/2003
LexicalTag
NON
ThesaurusID
NLM (2005)