PEX5 polyclonal antibody

• 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.

• Structure Composition :

  1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

• Product Name Alternative :

  PEX5; PBD2A; PBD2B; PTS1-BP; PTS1R; PXR1; RCDP5

• Swiss Prot :

  P50542

• Reactivity :

  Human, Mouse, Rat

• Immunogen :

  Recombinant fusion protein of human PEX5 (NP_000310.2) .

• Conjugation :

  Unconjugated

• Applications :

  WB, IF/ICC

• Dilution :

  WB,1:500 - 1:2000|IF/ICC,1:50 - 1:200

• Purity :

  The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE) .

• Modification :

  Unmodification

• Molecular Weight :

  71kDa

• Storage Conditions :

  Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.

• Notes :

  For research use only, not for use in diagnostic procedure.

• Specificity :

  Unmodification

• Host or Source :

  Rabbit

• CAS Number :

  9007-83-4