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Peroxin 5 rabbit pAb
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ES6957
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50 µL $148.00
100 µL $248.00
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Overview

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: -20°C/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)