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3.A.20.1.1
The peroxisomal importing translocon with receptors: Pex5p and Pex7p; and receptor facilitator: Pex4p. Peroxisomal biogenesis factor PEX26 is a membrane anchor for the multi-subunit PEX1-PEX6 protein complex that controls ubiquitination and dislocation of PEX5 cargo receptors for peroxisomal matrix protein import. PEX26 associates with the peroxisomal translocation pore via PEX14 (Guder et al. 2018). Luminal peroxisomal proteins are imported from the cytosol by mobile receptors, which then recycle back to the cytosol by a poorly understood process (Feng et al. 2022). Recycling requires receptor modification by a membrane-embedded ubiquitin ligase complex comprising three RING finger domain-containing proteins (Pex2, Pex10 and Pex12). Feng et al. 2022 reported a cryo-EM structure of the ligase complex, which together with biochemical and in vivo experiments reveals its function as a retrotranslocation channel for peroxisomal import receptors. Each subunit of the complex contributes five transmembrane segments that co-assemble into an open channel. The three ring finger domains form a cytosolic tower, with ring finger 2 (RF2) positioned above the channel pore. The N terminus of a recycling receptor is inserted from the peroxisomal lumen into the pore and monoubiquitylated by RF2 to enable extraction into the cytosol. If recycling is compromised, receptors are polyubiquitylated by the concerted action of RF10 and RF12 and degraded. This polyubiquitylation pathway also maintains the homeostasis of other peroxisomal import factors. Thus, a crucial step during peroxisomal protein import is clarified, and it explains why mutations in the ligase complex cause human disease (Feng et al. 2022).

Accession Number:O09012
Protein Name:Peroxisomal targeting signal 1 receptor
Length:639
Molecular Weight:70756.00
Species:Mus musculus (Mouse) [10090]
Location1 / Topology2 / Orientation3: Cytoplasm1 / Peripheral membrane protein2
Substrate protein polypeptide chain

Cross database links:

RefSeq: NP_033021.2    NP_787947.1   
Entrez Gene ID: 19305   
Pfam: PF00515   
KEGG: mmu:19305   

Gene Ontology

GO:0005778 C:peroxisomal membrane
GO:0005052 F:peroxisome matrix targeting signal-1 binding
GO:0000268 F:peroxisome targeting sequence binding
GO:0005515 F:protein binding
GO:0008022 F:protein C-terminus binding
GO:0048468 P:cell development
GO:0021795 P:cerebral cortex cell migration
GO:0021895 P:cerebral cortex neuron differentiation
GO:0007029 P:endoplasmic reticulum organization
GO:0006635 P:fatty acid beta-oxidation
GO:0007006 P:mitochondrial membrane organization
GO:0050905 P:neuromuscular process
GO:0001764 P:neuron migration
GO:0040018 P:positive regulation of multicellular organi...
GO:0006625 P:protein targeting to peroxisome
GO:0000038 P:very-long-chain fatty acid metabolic process

References (3)

[1] “A mouse model for Zellweger syndrome.”  Baes M.I.et.al.   9288097
[2] “The transcriptional landscape of the mammalian genome.”  Carninci P.et.al.   16141072
[3] “The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).”  The MGC Project Teamet.al.   15489334

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FASTA formatted sequence
1:	MAMRELVEGE CGGANPLMKL ATHFTQDKAL RQEGLRPGPW PPGASAAETV SKPLGVGTED 
61:	ELVSEFLQDQ NATLVSRAPQ TFKMDDLLAE MQEIEQSNFR QAPQRAPGVA DLALSENWAQ 
121:	EFLAAGDAVD VAQDYNETDW SQEFIAEVTD PLSVSPARWA EEYLEQSEEK LWLGDQEGSS 
181:	TADRWYDEYH PEEDLQHTAS DFVSKVDDPK LANSEFLKFV RQIGEGQVSL ESAAGSGGAQ 
241:	AEQWAAEFIQ QQGTSEAWVD QFTRPGNKIA ALQVEFERAK SAIESDVDFW DKLQAELEEM 
301:	AKRDAEAHPW LSDYDDLTSA SYDKGYQFEE ENPLRDHPQP FEEGLHRLEE GDLPNAVLLF 
361:	EAAVQQDPKH MEAWQYLGTT QAENEQELLA ISALRRCLEL KPDNRTALMA LAVSFTNESL 
421:	QRQACETLRD WLRYSPAYAH LVAPGEEGAT GAGPSKRILG SLLSDSLFLE VKDLFLAAVR 
481:	LDPTSIDPDV QCGLGVLFNL SGEYDKAVDC FTAALSVRPN DYLMWNKLGA TLANGNQSEE 
541:	AVAAYRRALE LQPGYIRSRY NLGISCINLG AHREAVEHFL EALNMQRKSR GPRGEGGAMS 
601:	ENIWSTLRLA LSMLGQSDAY GAADARDLSA LLAMFGLPQ