Preferred Name |
Uroporphyrinogen Decarboxylase |
Synonyms |
Carboxy-Lyase, Uroporphyrinogen |
Definitions |
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. |
ID |
http://purl.bioontology.org/ontology/MESH/D014575 |
altLabel |
Carboxy-Lyase, Uroporphyrinogen Uroporphyrinogen-III carboxy-lyase Uroporphyrinogen Carboxy Lyase Decarboxylase, Uroporphyrinogen Uroporphyrinogen III Decarboxylase Decarboxylase, Uroporphyrinogen III Uroporphyrinogen Carboxy-Lyase |
AN |
/defic permitted but consider also PORPHYRIA CUTANEA TARDA and HEPATOERYTHROPOIETIC PORPHYRIA |
AQL |
AD AE AI AN BI BL CF CH CL CS DE DF EC GE HI IM IP ME PD PH PK PO RE SD ST TO TU UL UR |
cui |
C0042085 |
DC |
1 |
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. |
DX |
19910101 |
HN |
91(76); was see under CARBOXY-LYASES 1976-90 |
Inverse of AQ |
http://purl.bioontology.org/ontology/MESH/Q000032 http://purl.bioontology.org/ontology/MESH/Q000138 http://purl.bioontology.org/ontology/MESH/Q000737 http://purl.bioontology.org/ontology/MESH/Q000493 http://purl.bioontology.org/ontology/MESH/Q000145 http://purl.bioontology.org/ontology/MESH/Q000506 http://purl.bioontology.org/ontology/MESH/Q000187 http://purl.bioontology.org/ontology/MESH/Q000096 http://purl.bioontology.org/ontology/MESH/Q000037 http://purl.bioontology.org/ontology/MESH/Q000134 http://purl.bioontology.org/ontology/MESH/Q000592 http://purl.bioontology.org/ontology/MESH/Q000528 http://purl.bioontology.org/ontology/MESH/Q000008 http://purl.bioontology.org/ontology/MESH/Q000235 http://purl.bioontology.org/ontology/MESH/Q000648 http://purl.bioontology.org/ontology/MESH/Q000276 http://purl.bioontology.org/ontology/MESH/Q000191 http://purl.bioontology.org/ontology/MESH/Q000633 http://purl.bioontology.org/ontology/MESH/Q000652 http://purl.bioontology.org/ontology/MESH/Q000009 http://purl.bioontology.org/ontology/MESH/Q000302 http://purl.bioontology.org/ontology/MESH/Q000502 http://purl.bioontology.org/ontology/MESH/Q000494 http://purl.bioontology.org/ontology/MESH/Q000266 http://purl.bioontology.org/ontology/MESH/Q000600 http://purl.bioontology.org/ontology/MESH/Q000378 http://purl.bioontology.org/ontology/MESH/Q000172 |
Inverse of RB |
EC 4.1.1.37 |
Machine permutation |
91; was see under CARBOXY-LYASES 1976-90 |
Mapped from | |
MDA |
19750725 |
MMR |
20130709 |
MN |
D08.811.520.224.125.900 |
notation |
D014575 |
prefLabel |
Uroporphyrinogen Decarboxylase |
RR |
9024-70-8 (CAS RN) |
TERMUI |
T042641 T042640 T551700 |
TH |
UNK (19XX) NLM (1976) NLM (2005) |
tui |
T116 T126 |
subClassOf |