The role of prohibitin membrane domains and of membrane lipids for mitochondrial gene expression
Mitochondria are dynamic cell organelles with essential catabolic and anabolic functions and critical roles in cell death pathways. Mitochondrial dysfunction contributes to ageing and acts causally in the pathogenesis of many neurodegenerative disorders. The group analyses the conserved proteolytic system of these organelles which ensures the quality control of mitochondrial proteins and regulates key steps during mitochondrial biogenesis. Processes associated with mitochondrial membranes are in focus of research. Using Saccharomyces cerevisiae and transgenic and conditional knockout mice as model systems, we analyse the function of conserved mitochondrial proteases on the molecular level as well as their physiological roles and their pathogenic relevance in mammals.
Main areas of research are:
- Proteolysis of inner membrane proteins
- Proteolytic control of mitochondrial morphology and biogenesis
- Mitochondrial proteases and neurodegeneration
- Role of prohibitins in the inner membrane
König T, Tröder SE, Bakka K, Korwitz A, Richter-Dennerlein R, Lampe PA, Patron M, Mühlmeister M, Guerrero-Castillo S, Brandt U, Decker T, Lauria I, Paggio A, Rizzuto R, Rugarli EI, De Stefani D, Langer T. (2016) The m-AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria. Mol Cell. 64(1):148-162. doi: 10.1016/j.molcel.2016.08.020.
Wai, T., García-Prieto, J., Baker, M.J., Merkwirth, C., Benit, P., Rustin, P., Rupérez, F.J., Barbas, C., Ibanez, B., and Langer, T. (2015). Imbalanced OPA1 processing and mitochondrial fragmentation cause heart failure in mice. Science, 2015 Dec 4;350(6265):aad0116. doi: 10.1126/science.aad0116.
Richter-Dennerlein R, Korwitz A, Haag M, Tatsuta T, Dargazanli S, Baker M, Decker T, Lamkemeyer T, Rugarli EI, Langer T. (2014) DNAJC19, a mitochondrial cochaperone associated with cardiomyopathy, forms a complex with prohibitins to regulate cardiolipin remodeling. Cell Metab. 1;20(1):158-71. doi: 10.1016/j.cmet.2014.04.016..
Max Planck Institute for Biology of Ageing
50931 Cologne, Germany