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).
Applications:Suitable for use in Western Blot, Immunohistochemistry, and ELISA.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemisty (paraffin): 1:100-1:300ELISA: 1:40,000Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, 0.5% BSA, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of human Peroxin 7. Species Crossreactivity: mouse and rat
Isotype：IgG
Calc Applications Abbrev：E IHC WB
Calc Crossreactivity：Hu Mo Rt
Immunogen：Synthetic peptide corresponding to aa180-260 of human Peroxin 7.
