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EC Number General Information Commentary Reference
Display the word mapDisplay the reaction diagram Show all sequences 3.4.24.64physiological function MPP plays an essential role in mitochondrial biogenesis and cell proliferation 709853
Display the word mapDisplay the reaction diagram Show all sequences 3.4.24.64physiological function primary enzyme responsible for the maturation of the vast majority of nuclear-encoded mitochondrial proteins, which is necessary for life at the cellular level. Analysis of lymphoblastoid cells and fibroblasts from patients homozygous for the PMPCA p.Ala377Thr, a mutation in the alpha subunit of mitochondrial processing peptidase and carriers demonstrate that the mutation impacts both the level of the alpha subunit encoded by PMPCA and the function of mitochondrial processing peptidase. This mutation impacts the maturation process of frataxin, the protein which is depleted in Friedreich ataxia 753119
Display the word mapDisplay the reaction diagram Show all sequences 3.4.24.64physiological function the mitochondrial processing protease is required in maturation of mitochondrial proproteins for removal of the N-terminal presequences 708076
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