PEX19 Antibody

• Background:

  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.

• NCBI Gene ID:

  5824

• Swiss Prot:

  P40855

• Accession Number:

  P40855

• Host:

  Rabbit

• Reactivity:

  Human

• 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.

• Clonality:

  Polyclonal

• Conjugation:

  Unconjugated

• Type:

  Primary Antibodies

• Field of Research:

  Signal Transduction

• Purification:

  This antibody is purified through a protein A column, followed by peptide affinity purification.

• Concentration:

  Batch dependent

• Homology:

  Predicted species reactivity based on immunogen sequence: Bovine, Rat

• Buffer:

  Supplied in PBS with 0.09% (W/V) sodium azide.

• Modification:

  None

• Shipping Conditions:

  Blue Ice

• Storage Conditions:

  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.

• Calculated Molecular Weight:

  33 kDa

• Fragment:

  Rabbit Ig

• Applications Notes:

  For WB starting dilution is: 1:1000

• Symbol:

  PEX19

• NCBI Official Name:

  Peroxisomal biogenesis factor 19

• NCBI Organism:

  Homo sapiens

• Background Reference 01:

  Mohamed, S., et al. Am. J. Med. Genet.152A (9), 2318-2321 (2010) :

• Background Reference 02:

  Schmidt, F., et al. J. Biol. Chem. 285 (33) :25410-25417 (2010)

• Background Reference 03:

  Schueller, N., et al. EMBO J. 29 (15) :2491-2500 (2010)

• Background Reference 04:

  Liu, Y., et al.Psychiatr Res (2010) In press :

• Other Product Names:

  Peroxisomal biogenesis factor 19, 33 kDa housekeeping protein, Peroxin-19, Peroxisomal farnesylated protein, PEX19, HK33, PXF

• Tested Applications:

  WB

• Protein ID:

  729723

• Physical Properties:

  Liquid