Anti-PMPCA Antibody

Raised in rabbit against the alpha subunit of the mitochondrial processing peptidase (MPP) complex, this polyclonal IgG reagent targets PMPCA, the subunit responsible for cleaving N-terminal presequences from nuclear-encoded precursors imported into the mitochondrial matrix. Working alongside the beta subunit encoded by PMPCB, MPP typically recognizes an arginine at the -2 position relative to its cleavage site, enabling maturation of hundreds of mitochondrial proteins.

The unconjugated antibody is supplied as 50 µl of polyclonal IgG and is validated for Western blot detection of endogenous PMPCA in lysates prepared from human, mouse, and rat samples. Recommended storage is at -20 °C in working aliquots, with freeze-thaw cycles kept to a minimum to preserve reactivity across repeated use.

Investigators studying mitochondrial biogenesis, protein import pathways, and the molecular basis of neurodegeneration will find this reagent useful. Biallelic PMPCA mutations cause autosomal recessive spinocerebellar ataxia type 2 (SCAR2), a childhood-onset cerebellar disorder mapped to chromosome 9q34.3, and the antibody supports studies of mutant protein stability as well as abnormal processing of client substrates such as frataxin (FXN). The PMPCA locus overlaps the INPP5E gene on the opposite strand, and the encoded 528-amino-acid protein shares 85.5% identity with the rat ortholog. Available through Antibodies.com for research use only.