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Details on Person UniProt:O60683-1 PEX10

Class:IdReferenceIsoform:401718
_chainChangeLogchain:1-326 added on Sat February 7 2015
_displayNameUniProt:O60683-1 PEX10
_timestamp2024-11-03 19:57:56
chainchain:1-326
checksum9CF2CE5E4C797799
commentFUNCTION E3 ubiquitin-protein ligase component of a retrotranslocation channel required for peroxisome organization by mediating export of the PEX5 receptor from peroxisomes to the cytosol, thereby promoting PEX5 recycling (PubMed:24662292). The retrotranslocation channel is composed of PEX2, PEX10 and PEX12; each subunit contributing transmembrane segments that coassemble into an open channel that specifically allows the passage of PEX5 through the peroxisomal membrane (By similarity). PEX10 also regulates PEX5 recycling by acting as a E3 ubiquitin-protein ligase (PubMed:24662292). When PEX5 recycling is compromised, PEX10 catalyzes polyubiquitination of PEX5 during its passage through the retrotranslocation channel, leading to its degradation (By similarity).CATALYTIC ACTIVITY S-ubiquitinyl-[E2 ubiquitin-conjugating enzyme]-L-cysteine + [acceptor protein]-L-lysine = [E2 ubiquitin-conjugating enzyme]-L-cysteine + N(6)-ubiquitinyl-[acceptor protein]-L-lysine.ACTIVITY REGULATION The E3 ubiquitin-protein ligase activity is stimulated by PEX12.PATHWAY Protein modification; protein ubiquitination.SUBUNIT Component of the PEX2-PEX10-PEX12 retrotranslocation channel, composed of PEX2, PEX10 and PEX12 (PubMed:24662292). Interacts with PEX19 (PubMed:10704444, PubMed:11390669).SUBCELLULAR LOCATION The three subunits of the retrotranslocation channel (PEX2, PEX10 and PEX12) coassemble in the membrane into a channel with an open 10 Angstrom pore (By similarity). The RING-type zinc-fingers that catalyze PEX5 receptor ubiquitination are positioned above the pore on the cytosolic side of the complex (By similarity).DISEASE 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.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the pex2/pex10/pex12 family.
created[InstanceEdit:400710] Schmidt, EE, 2009-03-25 05:33:35
descriptionrecommendedName: fullName evidence="20"Peroxisome biogenesis factor 10 ecNumber evidence="10"2.3.2.27 alternativeName: fullName evidence="20"Peroxin-10 alternativeName: Peroxisomal biogenesis factor 10 alternativeName: Peroxisome assembly protein 10 alternativeName: RING finger protein 69
geneNamePEX10
RNF69
identifierO60683
isoformParent
isSequenceChangedFALSE
keywordAlternative splicing
Disease variant
Membrane
Metal-binding
Peroxisome
Peroxisome biogenesis
Peroxisome biogenesis disorder
Protein transport
Proteomics identification
Reference proteome
Transferase
Transmembrane
Transmembrane helix
Transport
Ubl conjugation pathway
Zellweger syndrome
Zinc
Zinc-finger
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
namePEX10
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8996786] ENSEMBL:ENSG00000157911 PEX10 [Homo sapiens]
secondaryIdentifierPEX10_HUMAN
B3KWD8
Q5T095
Q9BW90
sequenceLength326
species[Species:48887] Homo sapiens
variantIdentifierO60683-1
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No pathways have been reviewed or authored by UniProt:O60683-1 PEX10 (401718)