Product Name :PEX5 mouse monoclonal antibody (KU)
Reactivity :Human, Dog, Rat, Monkey, Mouse
Application_all :WB 1:500~2000, IF 1:100, FLOW 1:100
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]
Product :1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2
Purification&Purity :The antibody was affinity-purified from mouse ascites fluids or tissue culture supernatant by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE).
Storage&Stability :Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Note :For research use only, not for use in diagnostic procedure.
Alternative Name :PBD2A; PBD2B; PTS1-BP; PTS1R; PXR1; RCDP5
Immunogen :Full length human recombinant protein of human PEX5 (NP_000310) produced in HEK293T cell.
Modification :Unmodification
