Project Summary
Most mitochondrial proteins contain targeting presequences that are proteolytically cleaved after import. We have shown that in human cells more than 100 of these proteins are subsequently processed by the evolutionary conserved aminopeptidase ICP55 and that this affects stability and efficiency of the respiratory chain complexes. In this project, we will investigate how ICP55-mediated amino-terminal processing affects the assembly and function of mitochondrial protein machineries.