PEX5 Mouse anti-Human, Clone: OTI6G8, liquid, TrueMAB
Mouse Monoclonal Antibody
Manufacturer: OriGene TA501406
DescriptionThe 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.
|PBS with 1% BSA, 50% glycerol and 0.02% sodium azide|
|PBD2A, PBD2B, PTS1-BP, PTS1R, PXR1, RCDP5|
|-20° C, Avoid Freeze/Thaw Cycles|
|Immunocytochemistry, Immunofluorescence, Western Blot|
|Full length human recombit protein of human PEX5 produced in HEK293T cell.|
The Fisher Scientific Encompass Program offers items which are not part of our distribution portfolio. These products typically do not have pictures or detailed descriptions. However, we are committed to improving your shopping experience. Please use the form below to provide feedback related to the content on this product.