Source: | Rabbit | Gene Id: | 5830 |
Isotype: | IgG | Swiss Prot: | P50542 |
purity: | Affinity purification |
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. |
Reactivity | Human, Mouse, Rat |
Tested applications | WB IHC |
Clonality | Polyclonal Antibody |
Calculated MW | 70kDa |
Recommended Dilutions |
WB 1:500 - 1:2000
IHC 1:50 - 1:200
|
Immunogen | A recombinant protein of human PEX5 |
Storage | Store at -20oC or -80oC. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
Synonym | PXR1, PBD2A, PBD2B, PTS1R, PTS1-BP |
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