| Product name: |
Peroxin 5 rabbit pAb |
| Reactivity: |
Human;Mouse |
| Alternative Names: |
PEX5; PXR1; Peroxisomal targeting signal 1 receptor; PTS1 receptor; PTS1R; PTS1-BP; Peroxin-5; Peroxisomal C-terminal targeting signal import receptor; Peroxisome receptor 1 |
| Source: |
Rabbit |
| Dilutions: |
WB 1:500-2000;IHC-p 1:50-300 |
| Immunogen: |
Synthesized peptide derived from Peroxin 5 . at AA range: 540-620 |
| Storage: |
-20C/1 year |
| Clonality: |
Polyclonal |
| Isotype: |
IgG |
| Concentration: |
1 mg/ml |
| Observed Band: |
70kD |
| GeneID: |
5830 |
| Human Swiss-Prot No: |
P50542 |
| Cellular localization: |
Cytoplasm . Peroxisome membrane ; Peripheral membrane protein. Its distribution appears to be dynamic. It is probably a cycling receptor found mainly in the cytoplasm and as well associated to the peroxisomal membrane through a docking factor (PEX13). |
| 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) |
