PMPCb is a member of the peptidase M16 family and encodes a protein with a zinc-binding motif. This protein is located in the mitochondrial matrix and catalyzes the cleavage of the leader peptides of precursor proteins newly imported into the mitochondria, though it only functions as part of a heterodimeric complex.In in vitro assays, MPPB bound frataxin, which was cleaved by the reconstituted MPP heterodimer. MPP cleavage of frataxin resulted in an intermediate form, comprising amino acids 41 to 210, which was processed further to the mature form. In vitro and in vivo experiments suggested that 2 C-terminal missense mutations found in FRDA patients, I151F and G130V, modulated interaction with MPP-beta, resulting in a slower maturation process at the normal cleavage site.
Bovine Mitochondrial-processing peptidase subunit beta (PMPCB) ELISA Kit employs a two-site sandwich ELISA to quantitate PMPCB in samples. An antibody specific for PMPCB has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and anyPMPCB present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for PMPCB is added to the wells. After washing, Streptavidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of PMPCB bound in the initial step. The color development is stopped and the intensity of the color is measured.
Bovine Mitochondrial-processing peptidase subunit beta (PMPCB) ELISA Kit listed herein is for research use only and is not intended for use in human or clinical diagnosis. Suggested applications of our products are not recommendations to use our products in violation of any patent or as a license. We cannot be responsible for patent infringements or other violations that may occur with the use of this product.
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