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Details on Person UniProt:P28328 PEX2

Class:IdReferenceGeneProduct:61538
_chainChangeLogchain:1-305 added on Fri February 6 2015
_displayNameUniProt:P28328 PEX2
_timestamp2024-11-03 19:53:09
chainchain:1-305
checksumF624F93C613FF2DB
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). PEX2 also regulates peroxisome organization by acting as a E3 ubiquitin-protein ligase (By similarity). PEX2 ubiquitinates PEX5 during its passage through the retrotranslocation channel: catalyzes monoubiquitination of PEX5 at 'Cys-11', a modification that acts as a signal for PEX5 extraction into the cytosol (By similarity). Required for pexophagy in response to starvation by mediating ubiquitination of peroxisomal proteins, such as PEX5 and ABCD3/PMP70 (PubMed:27597759). Also involved in the response to reactive oxygen species (ROS) by mediating 'Lys-48'-linked polyubiquitination and subsequent degradation of PNPLA2/ATGL, thereby regulating lipolysis (PubMed:34903883).CATALYTIC ACTIVITY [E2 ubiquitin-conjugating enzyme]-S-ubiquitinyl-L-cysteine + [acceptor protein]-L-cysteine = [E2 ubiquitin-conjugating enzyme]-L-cysteine + [acceptor protein]-S-ubiquitinyl-L-cysteine.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.PATHWAY Protein modification; protein ubiquitination.SUBUNIT Component of the PEX2-PEX10-PEX12 retrotranslocation channel, composed of PEX2, PEX10 and PEX12.INTERACTION 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).PTM Forms intramolecular and intermolecular disulfide bonds in response to reactive oxygen species (ROS), promoting higher stability.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.
descriptionrecommendedName: fullName evidence="20"Peroxisome biogenesis factor 2 ecNumber evidence="16"2.3.2.27 ecNumber evidence="2"2.3.2.36 alternativeName: fullName evidence="18"35 kDa peroxisomal membrane protein alternativeName: fullName evidence="20"Peroxin-2 alternativeName: Peroxisomal membrane protein 3 alternativeName: fullName evidence="19"Peroxisome assembly factor 1 shortName evidence="19"PAF-1 alternativeName: RING finger protein 72
geneNamePEX2
PAF1
PMP3
PMP35
PXMP3
RNF72
identifierP28328
isSequenceChangedFALSE
keywordAcetylation
Disease variant
Disulfide bond
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
namePEX2
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8990306] ENSEMBL:ENSG00000164751 PEX2 [Homo sapiens]
secondaryIdentifierPEX2_HUMAN
Q567S6
Q9BW41
sequenceLength305
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:8953913] PEX2 [peroxisomal membrane] [Homo sapiens]
[EntityWithAccessionedSequence:9625570] PEX2 [cytosol] [Homo sapiens]
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No pathways have been reviewed or authored by UniProt:P28328 PEX2 (61538)