Mitochondrial-processing peptidase subunit alpha (IPR037715)

Short name: PMPCA

Overlapping homologous superfamilies

Family relationships



Mitochondrial-processing peptidase subunit alpha (PMPCA or MPPA; MEROPS identifier M16.971) is a non-proteolytic component of the complex that removes the first transit peptide from the N terminus of proteins synthesized in the cytoplasm but destined for the mitochondrion. PMPCA is itself synthesized in the cytoplasm and has an N-terminal transit peptide. Mutations in PMPCA result in abnormal mitochondrial protein processing in patients with non-progressive cerebellar ataxia [PMID: 25808372].

GO terms

Biological Process

GO:0006627 protein processing involved in protein targeting to mitochondrion

Molecular Function

No terms assigned in this category.

Cellular Component

No terms assigned in this category.

Contributing signatures

Signatures from InterPro member databases are used to construct an entry.