BD-PE0210

PEX5 (14S6) Mouse Monoclonal antibody

常规抗体

PEX5

WB

Human,Dog,Mouse,Rat

PBS (pH 7.3) containing 1% BSA, 50% glycerol and 0.02% New type preservative N.

Full length human recombinant protein of human PEX5 (NP_000310) produced in HEK293T cell.

WB 1:2000, IF 1:100

69.7kDa

-20°C/1 year

Mouse

IgG1

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]

现货

Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)