Thermo Fisher Scientific PEX5 Monoclonal Antibody (OTI6C4), TrueMAB

Applications

Western Blot (WB)

• Tested Dilution: 1:2,000

Immunocytochemistry (ICC/IF)

• Tested Dilution: 1:100

Product Specifications

Target Information

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 one phenotype 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), Zellweger syndrome (ZWS), and may also be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.