|Other Names||Peroxisome biogenesis factor 1, Peroxin-1, Peroxisome biogenesis disorder protein 1, PEX1|
|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||Required for stability of PEX5 and protein import into the peroxisome matrix. Anchored by PEX26 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes.|
|Cellular Location||Cytoplasm. Peroxisome membrane. Note=Associated with peroxisomal membranes|
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Provided below are standard protocols that you may find useful for product applications.
This gene encodes a member of the AAA ATPase family, alarge group of ATPases associated with diverse cellular activities.This protein is cytoplasmic but is often anchored to a peroxisomalmembrane where it forms a heteromeric complex and plays a role inthe import of proteins into peroxisomes and peroxisome biogenesis.Mutations in this gene have been associated with complementationgroup 1 peroxisomal disorders such as neonataladrenoleukodystrophy, infantile Refsum disease, and Zellwegersyndrome.
Zhao, J., et al. BMC Med. Genet. 11, 96 (2010) :Yik, W.Y., et al. Hum. Mutat. 30 (3), E467-E480 (2009) :Gudbjartsson, D.F., et al. Nat. Genet. 40(5):609-615(2008)Matsuoka, S., et al. Science 316(5828):1160-1166(2007)Tamura, S., et al. J. Biol. Chem. 281(38):27693-27704(2006)
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