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peroxisome assembly protein 26; peroxin-26 (PEX26)
Function:
- probably required for protein import into peroxisomes
- anchors PEX1 & PEX6 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes.
- involved in the import of catalase & proteins containing a PTS2 target sequence, but not in import of proteins with a PTS1 target sequence
- interacts directly with PEX6 via its cytoplasmic domain
- interacts indirectly with PEX1, via its interaction with PEX6
Structure: belongs to the peroxin-26 family
Compartment:
- peroxisome membrane; single-pass type 2 membrane protein
Expression: -widely expressed
- highly expressed in kidney, liver, brain & skeletal muscles
- expressed at intermediate level in pancreas, placenta & heart
- weakly expressed in lung
Pathology:
- defects in PEX26 are the cause of peroxisome biogenesis disorder complementation group 8
- defects in PEX26 are a cause of
a) Zellweger syndrome
b) neonatal adrenoleukodystrophy
c) infantile Refsum disease
General
peroxisomal membrane protein (peroxin)
Properties
SIZE: entity length = 305 aa
MW = 34 kD
COMPARTMENT: peroxisome
MOTIF: transmembrane domain {247-267}
Peroxisomal matrix {268-305}
Database Correlations
OMIM correlations
MORBIDMAP 608666
UniProt Q7Z412
Pfam PF07163
Entrez Gene 55670
Kegg hsa:55670
References
- UniProt :accession Q7Z412
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=PEX26
- dbPEX, PEX gene Database
http://www.dbpex.org/home.php?select_db=PEX26