Our research interest is the diagnosis pathomechanism and treatment of inborn errors of metabolism. We focus on primary nuclear encoded mitochondrial diseases involving single respiratory chain complexes, defects in mitochondrial DNA replication, translation and mitochondrial morphology. We study tissue culture models, and primary fibroblasts from patients, using a variety of biochemical and molecular techniques. Over the years, we have investigated nucleotide metabolism, mtDNA translation, the effect of small molecules, antibiotics and recently, the link between complex IV deficiency and nuclear DNA damage. In collaboration with other researches we are also involved in studying the role and function of the mitochondrial respiratory chain in heat acclimatization, neurodegenerative diseases, diabetes and infertility.