PEX5 polyclonal, anti-human, mouse, rat

Background:
The product of this gene binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. 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. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular 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 neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified.

Synonyms: PBD2A, PBD2B, PTS1-BP, PTS1R, PXR1, RCDP5

Cellular Location: Cytoplasm Peripheral membrane protein Peroxisome membrane

Immunogen:
Recombinant protein of human PEX5

Gene Symbol: PEX5

Gene ID: 5830

Swiss prot: P50542

Calculated MW: 71kDa

Recommended dilution:
WB 1:500-2000, IHC 1:50-200, IF 1:50-200,

Purity:
Affinity purification

Storage Buffer:
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Storage:
Store at -20℃. Avoid freeze / thaw cycles.