|Application ||WB, E|
|Other Accession||Q9QYU1, Q3SZD1, NP_001180573.1, NP_002848.1|
|Calculated MW||32807 Da|
|Antigen Region||33-61 aa|
|Other Names||Peroxisomal biogenesis factor 19, 33 kDa housekeeping protein, Peroxin-19, Peroxisomal farnesylated protein, PEX19, HK33, PXF|
|Target/Specificity||This PEX19 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 33-61 amino acids from the N-terminal region of human PEX19.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||PEX19 Antibody (N-term) is for research use only and 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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Provided below are standard protocols that you may find useful for product applications.
This gene is necessary for early peroxisomal biogenesis. It acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. These disorders have at least 14 complementation groups, with more than one phenotype being observed for some complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of Zellweger syndrome (ZWS), as well as peroxisome biogenesis disorder complementation group 14 (PBD-CG14), which is also known as PBD-CGJ. Alternative splicing results in multiple transcript variants.
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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