Cow Mitochondrial-processing peptidase subunit beta (PMPCB) ELISA Kit
| Product Name | Cow Mitochondrial-processing peptidase subunit beta (PMPCB) ELISA Kit |
|---|---|
| Description | 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. This assay has high sensitivity and excellent specificity for detection of Bovine PMPCB. No significant cross-reactivity or interference between Bovine PMPCB and analogues was observed. |
| Synonyms | Beta-MPP, MPP11, MPPB, MPPP52, mitochondrial processing peptidase beta subunit |
| Method | Sandwich ELISA |
| Detection Range | Request Information |
| Sensitivity | Request Information |
| Reactivity | Bovine |
| Sample Types | Serum, Plasma, Other biological fluids. |
| Background | 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. |
| Supplier | Abebio |
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