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FLASH GENE
Symbol PEX3 contributors: mct - updated : 26-01-2018
HGNC name peroxisomal biogenesis factor 3
HGNC id 8858
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
  • integral peroxisomal membrane protein, the N terminus inside the peroxisome
  • the C terminus facing the peroxisome
  • two predicted transmembrane segments in the N terminus
  • HOMOLOGY
    Homologene
    FAMILY
    CATEGORY regulatory , structural protein
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,peroxisome
    intracellular,cytoplasm,organelle,endoplasmic reticulum
    intracellular,cytoplasm,cytosolic
    intracellular,nucleus,nucleoplasm
    text
  • PEX3 inserts co-translationally into the mammalian ER via the SEC61A1 translocon
  • basic FUNCTION
  • playing a selective, essential and direct role in peroxisomal membrane proteins (PMPs)import as a docking factor for PEX19
  • natural or artificial targeting of PEX3 to any endomembrane may initiate peroxisome formation and that also PEX3-containing mitochondria can serve as source for the de novo synthesis of peroxisomes
  • PEX3 and PEX19 are essential for peroxisome biogenesis
  • in peroxisomes, peroxins PEX3 and PEX19 are the principal protein components of the machinery required for early peroxisomal biogenesis
  • within the cell, PEX3 is stabilized by PEX19 preventing PEX3 aggregation
  • essential import receptors PEX3 and PEX14 target mitochondria, where they are selectively released into vesicular pre-peroxisomal structures
  • PEX19 and PEX3 play an indispensable role in peroxisomal membrane protein (PMP) biogenesis, peroxisome division, and inheritance
  • PEX3, an integral peroxisomal membrane protein, and the cytosolic shuttling receptor PEX19 are thought to be responsible for the early steps of peroxisome biogenesis and assembly of their matrix protein import machinery
  • CELLULAR PROCESS protein, post translation, targeting
    cell organization/biogenesis
    PHYSIOLOGICAL PROCESS
    text peroxisomal and endoplasmic reticulum targeting signal
    PATHWAY
    metabolism
    signaling
    a component
  • crucial function of the PEX3-PEX19 complex during formation of peroxisomes in peroxisome-deficient cells, pointing to a dual function of the PEX3-PEX19 interaction in peroxisome biogenesis
  • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • PEX3 requires PEX16 for ER location but is dispensable for the ER location of PEX16
  • membrane-anchored PEX3 serves as the receptor for cytosolic PEX19, which in turn recognizes newly synthesized peroxisomal membrane proteins
  • interaction between PEX3, which resides on the peroxisomal membrane, and PEX19, which resides in the cytosol, is crucial for peroxisome formation and the post-translational targeting of peroxisomal membrane proteins (PMPs)
  • PEX19 formed a complex with the peroxisomal Tail-anchored (TA) protein PEX26 in the cytosol and translocated it directly to peroxisomes by interacting with the peroxisomal membrane protein PEX3
  • PEX16 mediates the peroxisomal trafficking of two distinct peroxisomal membrane proteins, PEX3 and SLC25A17, via the ER
  • within the cell, PEX3 is stabilized by PEX19 preventing PEX3 aggregation
  • PEX19 bridged the interaction at the ER between PEX3 and RING-domain proteins, resulting in a ternary complex that was critical for the intra-ER sorting and subsequent budding of the RING-domain peroxins
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) ZWS6
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS