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Details on Person UniProt:P06132 UROD

Class:IdReferenceGeneProduct:53462
_chainChangeLogchain:1-367 added on Sat February 7 2015
_displayNameUniProt:P06132 UROD
_timestamp2025-05-21 21:42:03
chainchain:1-367
checksum840510B36CFC3856
commentFUNCTION 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
descriptionrecommendedName: fullName evidence="29"Uroporphyrinogen decarboxylase shortName: UPD shortName: URO-D ecNumber evidence="4 8 15"4.1.1.37
geneNameUROD
identifierP06132
isSequenceChangedFALSE
keyword3D-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
nameUROD
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8961135] ENSEMBL:ENSG00000126088 UROD [Homo sapiens]
secondaryIdentifierDCUP_HUMAN
A8K762
Q16863
Q16883
Q53YB8
Q53ZP6
Q6IB28
Q9BUZ0
sequenceLength367
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)