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FLASH GENE
Symbol PEX5 contributors: mct/pgu - updated : 25-01-2015
HGNC name peroxisomal biogenesis factor 5
HGNC id 9719
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • N terminus with seven diaromatic pentapeptide repeats (WxxxF/Y)
    • required for redirecting the peroxisome-associated peroxin back to the cytosol, mediating the interaction with the N-terminal region of PEX14 in mammals and with a natively unfolded N-terminal half, contributing to the interaction with cargo proteins
  • a peroxisomal targeting sequence type 1 (PTS1) or type 2 (PTS2)
  • a PEX14-binding site in PEX5, critical for matrix protein iImport into pPeroxisomes
  • seven tetrapeptide repeats (TPRs) organized into a ring-like structure in its C-terminal half (TPR-containing carboxy-terminal half mediate the interaction with the PTS1 sequence
    • conjugated GlycoP
    mono polymer heteromer
    HOMOLOGY
    Homologene
    FAMILY
  • peroxisomal targeting signal receptor family
    • CATEGORY receptor membrane , transport carrier
    SUBCELLULAR LOCALIZATION extracellular
        intracellular
    intracellular,cytoplasm,organelle,mitochondria
    intracellular,cytoplasm,organelle,lumen
    intracellular,cytoplasm,organelle,peroxisome
    intracellular,cytoplasm,organelle,Golgi
    intracellular,cytoplasm,organelle,endosome
    intracellular,cytoplasm,cytosolic
    text
  • translocated into the peroxisomal matrix and recycled to the cytosol concomittantly with formation of Pex4-Pex14p membrane complex
    • basic FUNCTION
  • plays an essential role in peroxisomal protein import
  • having a critical function in oligodendroglial peroxisomes for the support of axonal integrity
  • may act as a chaperone/holdase at least for some peroxisomal matrix proteins
  • PEX5 is the cytosolic receptor for peroxisome matrix proteins with peroxisome targeting signal type-1 and shuttles between the cytosol and peroxisomes
  • shuttling import receptor for peroxisomal matrix proteins, that is a redox-sensitive protein
  • matrix protein import requires a shuttle receptor protein, usually PEX5, that cycles through docking with the peroxisomal membrane
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    text peroxisomal cycling receptor
    PATHWAY
    metabolism
    signaling
    a component
  • complex of proteins with PEX10, PEX12 and others
    • INTERACTION
    DNA
    RNA
    small molecule nucleotide,
  • interaction with ATP predominantly needed downstream of the translocation step to reset the Pex5p-mediated transport system
    • protein
  • PEX12 acting downstream of receptor (PEX5) docking in peroxisomal matrix protein import
  • PEX10
  • PEX7
  • interacting with MYOC (associates with the myocilin C-terminus)
  • PEX14 binds the soluble receptors PEX5 and PEX19
  • binding of PEX5 to monomeric catalase potently inhibits its tetramerization, a property that depends on domains present in both the N- and C-terminal halves of PEX5
  • interaction with monomeric catalase (N-terminal domain of PEX14 disrupts this interaction)
  • ZFAND6 is a novel cofactor of PEX6 involved in the regulation of PEX5 export during peroxisome biogenesis
  • AAA peroxins are involved in the export from peroxisomes of PEX5
  • USP9X was found to be the most active deubiquitinase acting on ubiquitin-PEX5
  • USP9X participates in the PEX5-mediated peroxisomal protein import pathway
  • PEX5 binds proteins with peroxisomal targeting signal called PTS1 in the cytosol via its C-terminal tetratricopeptide domains and delivers them to the peroxisome lumen, where the receptor·cargo complex dissociates
  • matrix proteins associate with PEX5 in the cytosol and are translocated into the peroxisome lumen during the PEX5 cycle
  • N-terminal region in PEX26 acts as a scaffold protein to recruit PEX14·PEX5 complex together with PEX1·PEX6 complexes on peroxisomes
  • in the peroxisomal protein import, AAA peroxins modulate the interaction between PEX26 and PEX14 on peroxisome membrane as well as PEX5 oligomer in the cytosol
  • PEX1-PEX6 complexes facilitate the export of the peroxisomal matrix protein receptor PEX5 back into the cytosol after PEX5 has delivered its cargo to the peroxisome
    • cell & other
    REGULATION
    Other cytosolic cargo protein is inserted in the peroxisomal docking/translocation machinery, is then monoubiquitinated and thus gives access to the ATP-dependent dislocation of the ubiquitin-PEX5 conjugate back into the cytosol. Ubiquitin moiety is then removed and PEX5 is released
    ASSOCIATED DISORDERS
    corresponding disease(s) PBD3 , NALD2
    Susceptibility
    Variant & Polymorphism SNP
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS