PEX5 Mouse anti-Canine, Human, Clone: OTI1D3, liquid, TrueMAB
Mouse Monoclonal Antibody
Manufacturer: OriGene TA501389
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.|
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