Anti-PEX19 Antibody Picoband® 31.0550 Vervoeging

Peroxisomal biogenesis factor 19 is a protein that in humans is encoded by the PEX19 gene. 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.

PEX19

5824

P40855

Human

E.coli-derived human PEX19 recombinant protein (Position: Q51-A269) . Human PEX19 shares 91.8% and 94.5% amino acid (aa) sequence identity with mouse and rat PEX19, respectively.

Flow Cytometry

Immunogen affinity purified.

Liquid

1. Braun, A., Kammerer, S., Weissenhorn, W., Weiss, E. H., Cleve, H. Sequence of a putative human housekeeping gene (HK33) localized on chromosome 1. Gene 146: 291-295, 1994. 2. Gotte, K., Girzalsky, W., Linkert, M., Baumgart, E., Kammerer, S., Kunau, W.-H., Erdmann, R. Pex19p, a farnesylated protein essential for peroxisome biogenesis. Molec. Cell. Biol. 18: 616-628, 1998. 3. James, G. L., Goldstein, J. L., Pathak, R. K., Anderson, R. G. W., Brown, M. S. PxF, a prenylated protein of peroxisomes J. Biol. Chem. 269: 14182-14190, 1994.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

6

IgG

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.