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MIPEP 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.
Gene Name: | mitochondrial intermediate peptidase |
Family/Subfamily: | Protease , Metallopeptidase M3 |
Synonyms: | MIPEP, HMIP |
Target Sequences: | NM_005932 NP_005923.2 Q99797 |
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