UniProt:O00628 PEX7

chain
  • chain:1-323
checksum D405387F7F14B432
comment
  • FUNCTION Receptor required for the peroxisomal import of proteins containing a C-terminal PTS2-type peroxisomal targeting signal (PubMed:11931631, PubMed:22057399, PubMed:25538232, PubMed:9090381). Specifically binds to cargo proteins containing a PTS2 peroxisomal targeting signal in the cytosol (PubMed:11931631, PubMed:22057399, PubMed:25538232). Cargo protein-binding triggers interaction with PEX5 and formation of a ternary complex composed of PEX5 and PEX7 along with PTS2-containing cargo proteins, which is tranlocated into peroxisomes by passing through the PEX13-PEX14 docking complex (PubMed:11546814, PubMed:25538232).SUBUNIT Interacts with PEX5; interaction only takes place when PEX7 is associated with cargo proteins (PubMed:11546814, PubMed:25538232, PubMed:33389129). Interacts with VWA8 (PubMed:30204880).INTERACTION Translocated into the peroxisome matrix together with PTS2-containing cargo proteins and PEX5.ALTERNATIVE PRODUCTS Ubiquitous (PubMed:9090381). Highest expression in pancreas, skeletal muscle and heart (PubMed:9090381).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 WD repeat peroxin-7 family.
databaseName UniProt
dbId 61546
description
  • recommendedName: fullName evidence="13"Peroxisomal targeting signal 2 receptor shortName evidence="11"PTS2 receptor alternativeName: fullName evidence="13"Peroxin-7
displayName UniProt:O00628 PEX7
geneName
  • PEX7
  • PTS2R
identifier O00628
isSequenceChanged false
keyword
  • Alternative splicing
  • Cataract
  • Cytoplasm
  • Deafness
  • Disease variant
  • Ichthyosis
  • Peroxisome
  • Peroxisome biogenesis disorder
  • Protein transport
  • Proteomics identification
  • Reference proteome
  • Repeat
  • Retinitis pigmentosa
  • Rhizomelic chondrodysplasia punctata
  • Transport
  • WD repeat
moleculeType Protein
name
  • PEX7
schemaClass ReferenceGeneProduct
secondaryIdentifier
  • PEX7_HUMAN
  • C0H5X6
sequenceLength 323
stId uniprot:O00628

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