Molecular studies of mitochondrial gene expression

Mitochondria are the power plants of the eukaryotic cell and disturbed mitochondrial function causes a wide range of genetic diseases.

Although gene expression from mitochondrial DNA is of key importance for maintaining mitochondrial function, the molecular basis of mitochondrial gene expression is largely unexplored.

Transcription from mitochondrial DNA results in long polycistronic transcripts that need extensive processing and maturation to obtain the functional RNA units that are necessary for mitochondrial translation.

Here, we propose to determine the molecular basis of key steps in the mitochondrial gene expression machinery: DNA replication, initial RNA processing and mRNA maturation/stabilization. To this end, we will use a unique combination of biochemistry, biophysics and structural biology techniques.

Understanding these processes on the molecular level is critical for our understanding of fundamental aspects of mitochondrial gene expression and will, in the long term, enable new treatments for important mitochondrial diseases.