Mitochondrial-processing peptidase subunit beta is an enzyme that in humans is encoded by the PMPCBgene.
This gene 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.
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Gordon DM, Shi Q, Dancis A, Pain D (1999). "Maturation of frataxin within mammalian and yeast mitochondria: one-step processing by matrix processing peptidase.". Hum. Mol. Genet.8 (12): 2255–62. doi:10.1093/hmg/8.12.2255. PMID10545606.
Nagao Y, Kitada S, Kojima K, et al. (2000). "Glycine-rich region of mitochondrial processing peptidase alpha-subunit is essential for binding and cleavage of the precursor proteins.". J. Biol. Chem.275 (44): 34552–6. doi:10.1074/jbc.M003110200. PMID10942759.