Background:

PEX19 (peroxisomal biogenesis factor 19) 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 PEX19 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.

Classification:

Primary antibody

Alternative names:

PEX19; HK33; PXF; OK/SW-cl.22; Peroxisomal biogenesis factor 19; 33 kDa housekeeping protein; Peroxin-19; Peroxisomal farnesylated protein

Species reactivity:

Human

Host:

Rabbit

Isotype:

IgG

Immunogen:

The antiserum was produced against synthesized peptide derived from human PEX19. AA range:219-268

Concentration:

1 mg/ml

Molecular weight:

32807

Applications:

WB, IHC-p, ELISA

Storage:

-20°C for one year

More info:

Email: info@sobekbio.com

Orders:

Email: orders@sobekbio.com