|Other Names||Peroxisome assembly protein 26, Peroxin-26, PEX26|
|Target/Specificity||The synthetic peptide sequence used to generate the antibody AP14017b was selected from the C-term region of PEX26. A 10 to 100 fold molar excess to antibody is recommended. Precise conditions should be optimized for a particular assay.|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Probably required for protein import into peroxisomes. Anchors PEX1 and 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 and proteins containing a PTS2 target sequence, but not in import of proteins with a PTS1 target sequence.|
|Cellular Location||Peroxisome membrane; Single-pass type II membrane protein|
|Tissue Location||Widely expressed. Highly expressed in kidney, liver, brain and skeletal muscles. Expressed at intermediate level in pancreas, placenta and heart. Weakly expressed in lung|
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This gene belongs to the peroxin-26 gene family. It isprobably required for protein import into peroxisomes. It anchorsPEX1 and PEX6 to peroxisome membranes, possibly to form heteromericAAA ATPase complexes required for the import of proteins intoperoxisomes. Defects in this gene are the cause of peroxisomebiogenesis disorder complementation group 8 (PBD-CG8). PBD refersto a group of peroxisomal disorders arising from a failure ofprotein import into the peroxisomal membrane or matrix. The PBDgroup is comprised of four disorders: Zellweger syndrome (ZWS),neonatal adrenoleukodystrophy (NALD), infantile Refsum disease(IRD), and classical rhizomelic chondrodysplasia punctata (RCDP).Alternatively spliced transcript variants have been identified forthis gene.
Yik, W.Y., et al. Hum. Mutat. 30 (3), E467-E480 (2009) :Tamura, S., et al. J. Biol. Chem. 281(38):27693-27704(2006)Halbach, A., et al. J. Cell. Sci. 119 (PT 12), 2508-2517 (2006) :Furuki, S., et al. J. Biol. Chem. 281(3):1317-1323(2006)Weller, S., et al. Am. J. Hum. Genet. 76(6):987-1007(2005)
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