Impact of inaccurate mitochondrial translation on stress signaling and ageing
Biogenesis of mitochondrially encoded proteins
Mitochondria allow our cells to use oxidative phosphorylation as a highly efficient way to generate ATP. The membrane-bound respiratory chain complexes are composed of proteins from two different genetic origins, namely the nuclear and the mitochondrial DNA. The research of our group is focused on the synthesis and assembly of the mitochondrially encoded proteins. Defects in the expression of mitochondrial genes can cause or influence many human diseases including cancer, aging and mitochondrial disorders. However, surprisingly little is known about the molecular mechanisms underlying the biogenesis of mitochondrially encoded proteins. We employ a large variety of biochemical, cell and molecular biology methods to gain mechanistic insights into this fundamental process.
Ott M, Amunts A, Brown A. (2016) Organization and Regulation of Mitochondrial Protein Synthesis. Annu Rev Biochem. 85:77-101.
Kehrein K, Schilling R, Möller-Hergt BV, Wurm CA, Jakobs S, Lamkemeyer T, Langer T, Ott M. (2015) Organization of mitochondrial gene expression in two distinct ribosome-containing assemblies. Cell Rep 10(6):843–853.
Hildenbeutel M, Hegg EL, Stephan K, Gruschke S, Meunier B, Ott M. (2014) Assembly factors monitor sequential hemylation of cytochrome b to regulate mitochondrial translation. J Cell Biol. 205(4):511-24.