Diverse Functions of the Mitochondrial Presequence Processing Machinery

Project Summary

The mitochondrial proteome is mainly built by import of cytosolic precursor proteins. Upon import the majority of precursors require proteolytic processing by the presequence processing machinery, that ensures functional mature proteins. We found that the Oct1 protease forms different subassemblies with the processing machinery and with the mitoribosome. We aim to investigate the physiological role of the Oct1-mitoribosome interaction and also characterize the consequences of Oct1 patient mutations that show a decreased mitoribosome association.