|Other Names||Peroxisomal biogenesis factor 19, 33 kDa housekeeping protein, Peroxin-19, Peroxisomal farnesylated protein, PEX19, HK33, PXF|
|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||Necessary for early peroxisomal biogenesis. Acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Binds and stabilizes newly synthesized PMPs in the cytoplasm by interacting with their hydrophobic membrane-spanning domains, and targets them to the peroxisome membrane by binding to the integral membrane protein PEX3. Excludes CDKN2A from the nucleus and prevents its interaction with MDM2, which results in active degradation of TP53.|
|Cellular Location||Cytoplasm. Peroxisome membrane; Lipid- anchor; Cytoplasmic side. Note=Mainly cytoplasmic. Some fraction membrane-associated to the outer surface of peroxisomes|
|Tissue Location||Ubiquitously expressed. Isoform 1 is strongly predominant in all tissues except in utero where isoform 2 is the main form.|
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This gene is necessary for early peroxisomal biogenesis.It acts both as a cytosolic chaperone and as an import receptor forperoxisomal membrane proteins (PMPs). Peroxins (PEXs) are proteinsthat are essential for the assembly of functional peroxisomes. Theperoxisome biogenesis disorders (PBDs) are a group of geneticallyheterogeneous autosomal recessive, lethal diseases characterized bymultiple defects in peroxisome function. These disorders have atleast 14 complementation groups, with more than one phenotype beingobserved for some complementation groups. Although the clinicalfeatures of PBD patients vary, cells from all PBD patients exhibita defect in the import of one or more classes of peroxisomal matrixproteins into the organelle. Defects in this gene are a cause ofZellweger syndrome (ZWS), as well as peroxisome biogenesis disordercomplementation group 14 (PBD-CG14), which is also known asPBD-CGJ. Alternative splicing results in multiple transcriptvariants.
Mohamed, S., et al. Am. J. Med. Genet. A 152A (9), 2318-2321 (2010) :Schmidt, F., et al. J. Biol. Chem. 285(33):25410-25417(2010)Schueller, N., et al. EMBO J. 29(15):2491-2500(2010)Liu, Y., et al. J Psychiatr Res (2010) In press :Matsuzono, Y., et al. J. Biol. Chem. 281(1):36-42(2006)
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