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The mitochondrial flaws upon lack of PreP result from the buildup of presequence peptides that trigger feedback inhibition of MPP and accumulation of nonprocessed precursor proteins. Also, the mitochondrial advanced peptidase MIP that cleaves eight residues from a subset of precursors after MPP processing is affected upon loss of PreP recommending that PreP additionally degrades MIP generated octapeptides. Investigation of this PrePR183Q client mutation

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