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). 蛋白別名為:PEX7; PTS2R; Peroxisomal targeting signal 2 receptor; PTS2 receptor; Peroxin-7;基因ID為:5191;蛋白質ID:O00628
應用類型
WB,IHC-p,ELISA補充:最優的抗體稀釋比例需要基于客戶實驗進行優化.建議的起始稀釋比例如下: WB: 1:500-1:2000, IHC-p: 1:100-1:300, ELISA: 1:40000. Not yet tested in other applications.
免疫原
合成多肽:the Internal region of human Peroxin 7. at AA rangle: 180-260