Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Phe326 of human PEX5 protein.
Human, Mouse, Rat
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.
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. [provided by RefSeq, Oct 2008]
Peroxisomal Biogenesis Factor 5; Peroxisome Receptor 1; Peroxisomal C-Terminal Targeting Signal Import Receptor; Peroxisomal Targeting Signal 1 Receptor; Peroxisomal Import Receptor 5; PTS1 Receptor; Peroxin-5; PTS1-BP; PTS1R;