Background:

PEX7 (peroxisomal biogenesis factor 7) encodes the cytosolic receptor for the set of peroxisomal matrix enzymes targeted to the organelle by the peroxisome targeting signal 2 (PTS2). Defects in PEX7 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).

Classification:

Primary antibody

Alternative names:

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

Species reactivity:

Human;Mouse;Rat

Host:

Rabbit

Isotype:

IgG

Immunogen:

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

Concentration:

1 mg/ml

Molecular weight:

35892

Applications:

WB, IHC-p, ELISA

Storage:

-20°C for one year

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