peroxisomal biogenesis factor 3; peroxin-3; peroxisomal assembly protein PEX3 (PEX3)

Function

• role in peroxisome biosynthesis & integrity
• assembles membrane vesicles before the matrix proteins are translocated
• docking factor for PEX19, necessary for the import of peroxisomal membrane proteins in the peroxisomes
• binds to farnesylated PEX19

Structure

• 1st 45 amino acid residues targeting to peroxisomes
• belongs to the peroxin-3 family

Compartment

peroxisome membrane; multi-pass membrane protein

Expression

found in all examined tissues

Pathology

• defects in PEX3 are the cause of
  • peroxisome biogenesis disorder complementation group 12
  • Zellweger syndrome

More general terms

• peroxisomal membrane protein (peroxin)
• transmembrane 2 protein

Additional terms

• peroxisome

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=8504
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:8504
• OMIM: https://mirror.omim.org/entry/214100
• OMIM: https://mirror.omim.org/entry/601539
• OMIM: https://mirror.omim.org/entry/603164
• UniProt: http://www.uniprot.org/uniprot/P56589.html