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Details on Person UniProt:P06132 UROD
| Class:Id | ReferenceGeneProduct:53462 |
|---|---|
| _chainChangeLog | chain:1-367 added on Sat February 7 2015 |
| _displayName | UniProt:P06132 UROD |
| _timestamp | 2025-05-21 21:42:03 |
| chain | chain:1-367 |
| checksum | 840510B36CFC3856 |
| comment | FUNCTION Catalyzes the sequential decarboxylation of the four acetate side chains of uroporphyrinogen to form coproporphyrinogen and participates in the fifth step in the heme biosynthetic pathway (PubMed:11069625, PubMed:11719352, PubMed:14633982, PubMed:18004775, PubMed:21668429). Isomer I or isomer III of uroporphyrinogen may serve as substrate, but only coproporphyrinogen III can ultimately be converted to heme (PubMed:11069625, PubMed:11719352, PubMed:14633982, PubMed:21668429). In vitro also decarboxylates pentacarboxylate porphyrinogen I (PubMed:12071824).CATALYTIC ACTIVITY uroporphyrinogen III + 4 H(+) = coproporphyrinogen III + 4 CO2CATALYTIC ACTIVITY uroporphyrinogen I + 4 H(+) = coproporphyrinogen I + 4 CO2BIOPHYSICOCHEMICAL PROPERTIES Optimum pH is 6.8.PATHWAY Porphyrin-containing compound metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 4/4.SUBUNIT Homodimer.INTERACTION The disease is caused by variants affecting the gene represented in this entry.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the uroporphyrinogen decarboxylase family.ONLINE INFORMATION Uroporphyrinogen III decarboxylase entry |
| description | recommendedName: fullName evidence="29"Uroporphyrinogen decarboxylase shortName: UPD shortName: URO-D ecNumber evidence="4 8 15"4.1.1.37 |
| geneName | UROD |
| identifier | P06132 |
| isSequenceChanged | FALSE |
| keyword | 3D-structure Acetylation Cytoplasm Decarboxylase Direct protein sequencing Disease variant Heme biosynthesis Lyase Porphyrin biosynthesis Proteomics identification Reference proteome |
| modified | [InstanceEdit:9836292] Weiser, Joel, 2023-05-25 [InstanceEdit:9852000] Weiser, Joel, 2023-11-03 [InstanceEdit:9917590] Weiser, Joel, 2024-08-09 [InstanceEdit:9926675] Weiser, Joel, 2024-11-03 [InstanceEdit:9939033] Weiser, Joel, 2025-02-21 [InstanceEdit:9948485] Weiser, Joel, 2025-05-21 |
| name | UROD |
| referenceDatabase | [ReferenceDatabase:2] UniProt |
| referenceGene | [ReferenceDNASequence:8961135] ENSEMBL:ENSG00000126088 UROD [Homo sapiens] |
| secondaryIdentifier | DCUP_HUMAN A8K762 Q16863 Q16883 Q53YB8 Q53ZP6 Q6IB28 Q9BUZ0 |
| sequenceLength | 367 |
| species | [Species:48887] Homo sapiens |
| (referenceEntity) | [EntityWithAccessionedSequence:189427] UROD [cytosol] [Homo sapiens] [EntityWithAccessionedSequence:8961172] UROD [extracellular region] [Homo sapiens] |
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No pathways have been reviewed or authored by UniProt:P06132 UROD (53462)
