Contributes to metalloendopeptidase activity. Involved in protein processing involved in protein targeting to mitochondrion. Located in mitochondrion. Part of mitochondrial processing peptidase complex. Used to study multiple mitochondrial dysfunctions syndrome 6. Human ortholog(s) of this gene implicated in Alzheimer's disease and multiple mitochondrial dysfunctions syndrome 6. Orthologous to several human genes including PMPCB (peptidase, mitochondrial processing subunit beta).
SGD Description
Beta subunit of the mitochondrial processing protease (MPP); essential processing enzyme that cleaves the N-terminal targeting sequences from mitochondrially imported proteins