Product Datasheet  
PEX5 Antibody  
Catalog Number: 33042  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • Swiss-Prot:P50542
    NCBI Gene ID:5830
  • Form of Antibody：  
  • Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
  • Storage：  
  • Store at -20˚C
  • Immunogen：  
  • Recombinant protein of human PEX5.
  • reactivity：  
  • Human,Mouse,Rat
  • appl_detail：  
  • WB 1:500 - 1:2000
    IHC 1:50 - 1:200
    IF 1:50 - 1:200
  • other_names：  
  • PXR1; PBD2A; PBD2B; PTS1R; PTS1-BP
  • Purification：  
  • Antibodies were purified by affinity purification using immunogen.
  • Specificity：  
  • The antibody detects endogenous level of total PEX5 protein.
  • Applications：  
  • WB,IHC,IF
  • 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.



 
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