Department Larsson

Mitochondrial Biology

The group of Nils-Göran Larsson focuses on mitochondrial genetics and the impact of mitochondrial dysfunction on disease and ageing.

Mitochondrial dysfunction is heavily implicated in the ageing process. Ageing humans have increased levels of somatic mutations in the mitochondrial genome that tend to undergo clonal expansion to cause mosaic deficiencies in the respiratory chain. The oxidative phosphorylation (OXPHOS) system produces adenosine triphosphate (ATP), the universal source of energy in all tissues. Respiratory chain dysfunction, and thus insufficient supply of ATP, can cause a variety of phenotypes associated with ageing, age-related- and mitochondrial diseases. [more...]


Selected publications

Ross, J.M., Stewart, J.B., Hagström, E., Brené, S., Mourier, A., Coppotelli, G., Freyer, C., Lagouge, M., Hoffer, B.J., Olson, L., and Larsson, N.-G. (2013). Germline mitochondrial DNA mutations aggravate ageing and can impair brain development. Nature. Epub ahead of print.

Freyer, C., Cree, L.M., Mourier, A., Stewart, J.B., Koolmeister, C., Milenkovic, D., Wai, T., Floros, V.I., Hagström, E., Chatzidaki, E.E., et al. (2012). Variation in germline mtDNA heteroplasmy is determined prenatally but modified during subsequent transmission. Nat Genet 44, 1282-1285.

Kukat, C., Wurm, C.A., Spåhr, H., Falkenberg, M., Larsson, N.-G., and Jakobs, S. (2011). Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of mtDNA. Proc Natl Acad Sci USA 108, 13534-13539.

Larsson, N.-G. (2010). Somatic mitochondrial DNA mutations in mammalian aging. Annu Rev Biochem 79, 683-706.

Metodiev, M.D., Lesko, N., Park, C.B., Cámara, Y., Shi, Y., Wibom, R., Hultenby, K., Gustafsson, C.M., and Larsson, N.-G. (2009). Methylation of 12S rRNA is necessary for in vivo stability of the small subunit of the mammalian mitochondrial ribosome. Cell Metab 9, 386-397.

Trifunovic, A., Wredenberg, A., Falkenberg, M., Spelbrink, J.N., Rovio, A.T., Bruder, C.E., Bohlooly-Y, M., Gidlöf, S., Oldfors, A., Wibom, R., et al. (2004). Premature ageing in mice expressing defective mitochondrial DNA polymerase. Nature 429, 417-423.


Third-party funding

  • 2013 | DFG
    CECAD: EXC 229 - to support collaborative research

  • 2011-2016 | European Research Council (ERC) FP7, Advanced Grant
    Genemit: Regulation of gene expression in mammalian mitochondria

  • 2009-2016 | DFG
    SFB829: Molecular mechanisms regulating skin homeostasis

Assistance

Angela Maiburg

Tel.: +49 (0)221 37970-202
Fax: +49 (0)221 37970 -88-202

E-mail: AMaiburg@age.mpg.de