| Modification Notes |
MIPEP (Mitochondrial intermediate peptidase) is a 713 amino acid protein that performs the final step in processing nuclear-encoded proteins that are targeted the mitochondrial inner membrane or matrix. MIPEP is primarily involved in the maturation of oxidative phosphorylation-related proteins and is accordingly expressed at highest levels in tissues that have high oxygen consumption, such as skeletal muscle, brain, heart and pancreas. Significantly, human MIPEP is the functional homolog of yeast YMIP, a protein that promotes mitochondrial iron uptake and is regulated by the yeast homolog of frataxin, which is required for mitochondrial iron efflux. Due to the likely regulatory effect that human frataxin has on it, MIPEP may be implicated in the clinical manifestations of Friedreich ataxia, an autosomal recessive neurodegenerative disease caused by deficiency of frataxin. |