Mitochondrial Biogenesis and Assembly of Membrane Protein Complexes

We analyze the molecular mechanisms of protein transport across the mitochondrial membranes in order to find out how multi-protein complexes in the outer (TOM) and inner (TIM) membrane mediate this task. This transport process is regulated by intrinsic signals of the precursor proteins that are recognized by the TOM and TIM complexes in the membranes. For our analyses we utilize genetically accessible model systems, in vitro import analyses, and biochemical techniques to address the signal-mediated vectorial transfer of the unfolded polypeptide chain through the transport machineries.

Another aspect of our work addresses how multi-protein complexes in the inner membrane are assembled from nuclear-encoded subunits that are imported into mitochondria and mitochondria-encoded subunits exported from the matrix. The studies aim to understand the molecular mechanisms underlying assembly processes in order to understand how defects in these processes lead to neuromuscular disorders. We utilize different cell systems and use genetic as well as biochemical approaches.