The product of this gene performs the final step in processing a specific class of nuclear-encoded proteins targeted to the mitochondrial matrix or inner membrane. This protein is primarily involved in the maturation of oxidative phosphorylation (OXPHOS)-related proteins. This gene may contribute to the functional effects of frataxin deficiency and the clinical manifestations of Friedreich ataxia. [provided by RefSeq, Jul 2008]
Function:
Cleaves proteins, imported into the mitochondrion, to their mature size.
Subcellular Location:
Mitochondrion matrix.
Similarity:
Belongs to the peptidase M3 family.
SWISS:
Q99797
Gene ID:
4285
Database links:
Entrez Gene: 4285 Human
Omim: 602241 Human
SwissProt: Q99797 Human
Unigene: 507498 Human
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