Mitochondrial biology

Mitochondria, the power stations of a cell, have their own genome, mitochondrial DNA (mtDNA), which is essential for the healthy functions of the cells. Various genetic diseases are caused by increasing damage or loss of mitochondrial DNA in body tissues of patients; these diseases are often devastating. For unknown reasons only some tissues, often muscle or brain, are affected, while others do not show any symptom. Our group investigates how mitochondria function and how mtDNA is maintained and duplicated in different organs such as muscle, liver and brain. We try to explain the differences in these tissues and find the factors that are involved in damage, protection and repair of mitochondrial DNA. Thus we aim to explain how the mtDNA diseases are caused and why only some tissues are affected. In the long run we hope to find means to prevent, treat or alleviate the symptoms of these diseases. We are also interested in outstanding questions in evolutionary biology related to mitochondria, such as how mitochondria interact with the nucleus, possibly enforcing species boundaries.