Peroxin 7 rabbit pAb

• Background :

  This gene encodes the cytosolic receptor for the set of peroxisomal matrix enzymes targeted to the organelle by the peroxisome targeting signal 2 (PTS2) . Defects in this gene cause peroxisome biogenesis disorders (PBDs), which are characterized by multiple defects in peroxisome function. There are at least 14 complementation groups for PBDs, with more than one 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 have been associated with PBD complementation group 11 (PBD-CG11) disorders, rhizomelic chondrodysplasia punctata type 1 (RCDP1), and Refsum disease (RD) . [provided by RefSeq, Oct 2008]

• Description :

  This gene encodes the cytosolic receptor for the set of peroxisomal matrix enzymes targeted to the organelle by the peroxisome targeting signal 2 (PTS2). Defects in this gene cause peroxisome biogenesis disorders (PBDs), which are characterized by multiple defects in peroxisome function. There are at least 14 complementation groups for PBDs, with more than one 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 have been associated with PBD complementation group 11 (PBD-CG11) disorders, rhizomelic chondrodysplasia punctata type 1 (RCDP1), and Refsum disease (RD). [provided by RefSeq, Oct 2008],

• UniProt :

  O00628

• Swiss Prot :

  O00628

• Reactivity :

  Human; Mouse; Rat

• Immunogen :

  The antiserum was produced against synthesized peptide derived from human PEX7. AA range:204-253

• Clonality :

  Polyclonal

• Source :

  Rabbit

• Applications :

  WB; IHC; IF; ELISA

• Concentration :

  1 mg/ml

• Dilution :

  Western Blot: 1/500 - 1/2000. Immunohistochemistry: 1/100 - 1/300. ELISA: 1/40000. Not yet tested in other applications.

• Molecular Weight :

  40kD

• Storage Conditions :

  -20°C/1 year

• Observed Molecular Weight :

  40kD

• Fragment :

  IgG

• Subcellular Location :

  Peroxisome . Cytoplasm .

• Other Product Names :

  PEX7; PTS2R; Peroxisomal targeting signal 2 receptor; PTS2 receptor; Peroxin-7

• Gene ID (Human) :

  5191