商品介绍

gene_symbol：PEX5
Synonyms：PBD2A; PBD2B; PTS1-BP; PTS1R; PXR1; RCDP5
clone_name：OTI6G8
immunogen：Full length human recombinant protein of human PEX5 (NP_000310) produced in HEK293T cell.
predicted_size：69.7 kDa
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. [provided by RefSeq]
buffer：PBS (pH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.
purification：Purified from mouse ascites fluids or tissue culture supernatant by affinity chromatography (protein A/G)
isotype：IgG1
host：Mouse
applications：IF, WB
Recommend Dilution：WB 1:2000, IF 1:100
reactivities：Human, Mouse, Rat
storage：Store at -20°C as received.