Anti-PEX19 Antibody Picoband®

SKU: A03186-2

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human

Applications: WB,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: PEX19

Specificity:

Background: 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.

Immunogen: 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.

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 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.

Uniprot ID: P40855

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity:

Isotype: IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 38 kDa

Calculated Molecular Weight:

Gene ID: 5824

Protein Name: Peroxisomal biogenesis factor 19

Gene Full Name: peroxisomal biogenesis factor 19

Synonyms: 33 kDa housekeeping protein; D1S2223E; HK33; Peroxin 19; PEX19; PMP1; PMPI; PXF; PXMP1