Official Full Name
mitochondrial intermediate peptidase
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.
MIPEP; mitochondrial intermediate peptidase; MIP; HMIP; MIP, HMIP