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.1) Mohamed, S., et al. Am. J. Med. Genet.152A (9), 2318-2321 (2010) :
Immunogen
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.
Western blot analysis in Hela cell line lysates (35ug/lane).
Application Notes
For WB starting dilution is: 1:1000:
Additional Information
Form
Liquid
Storage Instructions
Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.