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Our main research aim is to understand how changes on mtDNA affect mitochondrial function and cell metabolism.

Nuclear and mitochondrial genomes are subjected to dynamic modifications. These modifications are taken care of by DNA glycosylases.

We have found that modifications in the DNA in nucleus and mitochondria serve distinct roles in cellular regulation of metabolism.

Thus, we believe that modifications in mitochondrial DNA (mtDNA) represent intracellular signals that are recognized by DNA glycosylases and metabolic factors in the mitochondrial nuelcoid.

We combine our developed high-resolution methods to quantify site-specific DNA modifications with mitochondrial and metabolic measurements to study the mtDNA- metabolic relation.


  • Metabolism of branched-chain amino acids. This essential catabolic pathway is blocked and cause pathology in MSUD (maple syrup urine disease) patients. On the other hand, the pathway is increased and associated with tumorigenesis in Pancreatic cancer. mtDNA is molecularly linked to this pathway via the core unit (E2b) of branched-chain ketoacid dehydrogenase. We study the regulation of energy metabolism in these diseases.
  • Mitochondrial characterization of human Retinal Epithelial Pigmental cells (primary cells and iPS-derived). We use mtDNA integrity as quality marker for cell homeostasis.
  • Mitochondrial dysfunction in Huntingtons Disease. We focus on genomic alteration and mtDNA heteroplasmy in patients and mouse/pig models of HD, to investigate the impact on mitochondrial function.
  • Branched-chain fatty acids are derived from branched-chain fatty acids, and are produced by microbiota. We investigate the impact of these fatty acids on mitochondrial adaption in colonocytes.


  • Bjørn Dalhus, University of Oslo, Oslo
  • Goran Petrovski, Centre of Eye Research, University of Oslo and Oslo University Hospital
  • Hana Hansikova, Charles University in Prague and General University Hospital in Prague, Czech Republic
  • Katja Benedicte Prestø Elgstøen, Oslo University Hospital, Oslo
  • Knut Rudi, Norwegian University of Life Science
  • Lyudmila Rachek, UoSouth Alabama College of Medicine
  • Magnar Bjørås, University of Oslo
  • Zdenka Ellederova & Jan Motlik, Institute of Animal Physiology and Genetics, Libechov, Czech Republic
Published Apr. 27, 2017 12:51 PM - Last modified Jan. 28, 2021 6:35 PM


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