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.
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Protein Aliases: MIP; Mitochondrial intermediate peptidase
Gene Aliases: 5730405E07Rik; HMIP; MIP; MIPEP
Molecular Function: metalloprotease