We are interested in the diverse aspects of mitochondrial protein biogenesis and how cells maintain proteostasis following proteotoxic damage. We combine classical biochemical and cell biological approaches with global proteomic and lipidomic analyses using our main model systems, yeast and human cell lines.

Mitochondrial biogenesis and
protein quality control

Mitochondria import 99% of their proteome as precursor proteins from the cytosol. We are investigating the pathways these precursors take from their synthesis on cytosolic ribosomes to their final destination within the mitochondria. In particular, we focus on the maturation process by proteolytic removal of the targeting signals, which is carried out by highly conserved presequence proteases and essential for the generation and maintenance of a functional mitochondrial proteome.

How does a cell sense mitochondrial defects and what are the protective mechanisms that maintain mitochondrial proteostasis? We have established mitochondrial stress models based on patient mutations that trigger mitochondrial proteotoxic stress to identify the different levels of protection that cells use to overcome mitochondrial proteostasis defects.

Mitochondrial dysfunction and protective cellular responses