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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
a) peroxisome biogenesis disorder complementation group 12
b) Zellweger syndrome
Related
peroxisome
General
peroxisomal membrane protein (peroxin)
transmembrane 2 protein
Properties
SIZE: entity length = 373 aa
MW = 42 kD
COMPARTMENT: peroxisome
MOTIF: cytoplasmic domain {1-15}
transmembrane domain {16-36}
exoplasmic loop {37-116}
transmembrane domain {117-140}
MOTIF: PEX19 interaction {120-136}
cytoplasmic domain {141-373}
Database Correlations
OMIM correlations
UniProt P56589
Pfam PF04882
Entrez Gene 8504
Kegg hsa:8504
References
- UniProt :accession P56589
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=PEX3