UniProt ID: E7F4V8 |
FUNCTION: 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 (By similarity). 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. PEX2 also regulates peroxisome organization by acting as a E3 ubiquitin-protein ligase (By similarity). {ECO:0000250|UniProtKB:P28328, ECO:0000250|UniProtKB:P32800}. CATALYTIC ACTIVITY: Reaction=[E2 ubiquitin-conjugating enzyme]-S-ubiquitinyl-L-cysteine + [acceptor protein]-L-cysteine = [E2 ubiquitin-conjugating enzyme]-L- cysteine + [acceptor protein]-S-ubiquitinyl-L-cysteine.; EC=2.3.2.36; Evidence={ECO:0000250|UniProtKB:P32800}; CATALYTIC ACTIVITY: Reaction=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.; EC=2.3.2.27; Evidence={ECO:0000250|UniProtKB:P28328}; PATHWAY: Protein modification; protein ubiquitination. {ECO:0000250|UniProtKB:P28328}. SUBUNIT: Component of the PEX2-PEX10-PEX12 retrotranslocation channel. {ECO:0000250|UniProtKB:P28328}. SUBCELLULAR LOCATION: Peroxisome membrane {ECO:0000250|UniProtKB:P28328}; Multi-pass membrane protein {ECO:0000255}. DOMAIN: The three subunits of the retrotranslocation channel (PEX2, PEX10 and PEX12) coassemble in the membrane into a channel with an open 10 Angstrom pore. The RING-type zinc-fingers that catalyze PEX5 receptor ubiquitination are positioned above the pore on the cytosolic side of the complex. {ECO:0000250|UniProtKB:G2Q1C9}. DISRUPTION PHENOTYPE: Mutant fishes show a loss of peroxisomal structure, associated with high-mortality (PubMed:34016526). Defects are similar to human Zellweger syndrome (ZS), characterized by developmental abnormalities in many organs, organ-specific accumulation and reduction of distinct fatty acid species, such as an accumulation of ultra-very-long-chain polyunsaturated fatty acids (ultra-VLC-PUFAs) (PubMed:34016526). {ECO:0000269|PubMed:34016526}. SIMILARITY: Belongs to the pex2/pex10/pex12 family. {ECO:0000305}. |
UniProt ID: A0A9B7QDH4 |
CATALYTIC ACTIVITY: Reaction=[E2 ubiquitin-conjugating enzyme]-S-ubiquitinyl-L-cysteine + [acceptor protein]-L-cysteine = [E2 ubiquitin-conjugating enzyme]-L- cysteine + [acceptor protein]-S-ubiquitinyl-L-cysteine.; EC=2.3.2.36; Evidence={ECO:0000256|ARBA:ARBA00034438}; PATHWAY: Protein modification; protein ubiquitination. {ECO:0000256|ARBA:ARBA00004906}. SUBCELLULAR LOCATION: Membrane {ECO:0000256|ARBA:ARBA00004141}; Multi- pass membrane protein {ECO:0000256|ARBA:ARBA00004141}. Peroxisome membrane {ECO:0000256|ARBA:ARBA00004585}; Multi-pass membrane protein {ECO:0000256|ARBA:ARBA00004585}. SIMILARITY: Belongs to the pex2/pex10/pex12 family. {ECO:0000256|ARBA:ARBA00008704}. |
This information was provided by UniProt through a collaboration with ZFIN. (1) |