Publications

Krüger, A.; Remes, C.; Shiriaev, D. I.; Liu, Y.; Spåhr, H.; Wibom, R.; Atanassov, I.; Nguyen, M. D.; Cooperman, B. S.; Rorbach, J.: Human mitochondria require mtRF1 for translation termination at non-canonical stop codons. Nature Communications 14, 30 (2023)

Burr, S. P.; Klimm, F.; Glynos, A.; Prater, M.; Sendon, P.; Nash, P.; Powell, C. A.; Simard, M.-L.; Bonekamp, N. A.; Charl, J. et al.; Diaz, H.; Bozhilova, L. V.; Nie, Y.; Zhang, H.; Frison, M.; Falkenberg, M.; Jones, N.; Minczuk, M.; Stewart, J. B.; Chinnery, P. F.: Cell lineage-specific mitochondrial resilience during mammalian organogenesis. Cell (2023)

Milenkovic, D.; Misic, J.; Hevler, J. F.; Molinié, T.; Chung, I.; Atanassov, I.; Li, X.; Filograna, R.; Mesaros, A.; Mourier, A. et al.; Heck, A. J.R.; Hirst, J.; Larsson, N.-G.: Preserved respiratory chain capacity and physiology in mice with profoundly reduced levels of mitochondrial respirasomes. Cell Metabolism (2023)

Milenkovic, D.; Sanz-Moreno, A.; Calzada-Wack, J.; Rathkolb, B.; Veronica Amarie, O.; Gerlini, R.; Aguilar-Pimentel, A.; Misic, J.; Simard, M.-L.; Wolf, E. et al.; Fuchs, H.; Gailus-Durner, V.; de Angelis, M. H.; Larsson, N.G.: Mice lacking the mitochondrial exonuclease MGME1 develop inflammatory kidney disease with glomerular dysfunction. PLoS Genet 18 (5), p. e1010190 (2022)

Miranda, M.; Bonekamp, N. A.; Kühl, I.: Starting the engine of the powerhouse: mitochondrial transcription and beyond. Biol Chem (2022)

Mennuni, M.; Filograna, R.; Felser, A.; Bonekamp, N. A.; Giavalisco, P.; Lytovchenko, O.; Larsson, N.G.: Metabolic resistance to the inhibition of mitochondrial transcription revealed by CRISPR-Cas9 screen. EMBO Rep 23 (1), p. e53054 (2022)

Misic, J.; Milenkovic, D.; Al-Behadili, A.; Xie, X.; Jiang, M.; Jiang, S.; Filograna, R.; Koolmeister, C.; Siira, S. J.; Jenninger, L. et al.; Filipovska, A.; Clausen, A. R.; Caporali, L.; Valentino, M. L.; La Morgia, C.; Carelli, V.; Nicholls, T. J.; Wredenberg, A.; Falkenberg, M.; Larsson, N.G.: Mammalian RNase H1 directs RNA primer formation for mtDNA replication initiation and is also necessary for mtDNA replication completion. Nucleic Acids Res 50 (15), pp. 8749 - 8766 (2022)

Rossetti, G.; Ermer, J. A.; Stentenbach, M.; Siira, S. J.; Richman, T. R.; Milenkovic, D.; Perks, K. L.; Hughes, L. A.; Jamieson, E.; Xiafukaiti, G. et al.; Ward, N. C.; Takahashi, S.; Gray, N.; Viola, H. M.; Hool, L. C.; Rackham, O.; Filipovska, A.: A common genetic variant of a mitochondrial RNA processing enzyme predisposes to insulin resistance. Sci Adv 7 (39), p. eabi7514 (2021)

Bonekamp, N.; Jiang, M.; Motori, E.; Garcia Villegas, R.; Koolmeister, C.; Atanassov, I.; Mesaros, A.; Park, C. B.; Larsson, N.G.: High levels of TFAM repress mammalian mitochondrial DNA transcription in vivo. Life Sci Alliance 4 (11), p. e202101034 (2021)

Jiang, M.; Xie, X.; Zhu, X.; Jiang, S.; Milenkovic, D.; Misic, J.; Shi, Y.; Tandukar, N.; Li, X.; Atanassov, I. et al.; Jenninger, L.; Hoberg, E.; Albarran-Gutierrez, S.; Szilagyi, Z.; Macao, B.; Siira, S. J.; Carelli, V.; Griffith, J. D.; Gustafsson, C. M.; Nicholls, T. J.; Filipovska, A.; Larsson, N.G.; Falkenberg, M.: The mitochondrial single-stranded DNA binding protein is essential for initiation of mtDNA replication. Sci Adv 7 (27), p. eabf8631 (2021)

Sprenger, H.-G.; MacVicar, T.; Bahat, A.; Fiedler, K. U.; Hermans, S.; Ehrentraut, D.; Ried, K.; Milenkovic, D.; Bonekamp, N.; Larsson, N.G. et al.; Nolte, H.; Giavalisco, P.; Langer, T.: Cellular pyrimidine imbalance triggers mitochondrial DNA–dependent innate immunity. Nature Metabolism 3 (5), pp. 636 - 650 (2021)

Bonekamp, N.; Peter, B.; Hillen, H. S.; Felser, A.; Bergbrede, T.; Choidas, A.; Horn, M.; Unger, A.; Di Lucrezia, R.; Atanassov, I. et al.; Li, X.; Koch, U.; Menninger, S.; Boros, J.; Habenberger, P.; Giavalisco, P.; Cramer, P.; Denzel, M. S.; Nussbaumer, P.; Klebl, B.; Falkenberg, M.; Gustafsson, C. M.; Larsson, N.G.: Small-molecule inhibitors of human mitochondrial DNA transcription. Nature 588 (7839), pp. 712 - 716 (2020)

Basu, S.; Xie, X.; Uhler, J. P.; Hedberg-Oldfors, C.; Milenkovic, D.; Baris, O. R.; Kimoloi, S.; Matic, S.; Stewart, J.; Larsson, N. G. et al.; Wiesner, R. J.; Oldfors, A.; Gustafsson, C. M.; Falkenberg, M.; Larsson, E.: Accurate mapping of mitochondrial DNA deletions and duplications using deep sequencing. PLoS Genet 16 (12), p. e1009242 (2020)

Bergami, M.; Motori, E.: Reweaving the Fabric of Mitochondrial Contact Sites in Astrocytes. Front Cell Dev Biol 8, p. 592651 (2020)

Motori, E.; Atanassov, I.; Kochan, S. M. V.; Folz-Donahue, K.; Sakthivelu, V.; Giavalisco, P.; Toni, N.; Puyal, J.; Larsson, N.G.: Neuronal metabolic rewiring promotes resilience to neurodegeneration caused by mitochondrial dysfunction. Sci Adv 6 (35), p. eaba8271 (2020)

Sharma, S.; Koolmeister, C.; Tran, P.; Nilsson, A. K.; Larsson, N.G.; Chabes, A.: Proofreading deficiency in mitochondrial DNA polymerase does not affect total dNTP pools in mouse embryos. Nat Metab 2 (8), pp. 673 - 675 (2020)

Alsina, D.; Lytovchenko, O.; Schab, A.; Atanassov, I.; Schober, F. A.; Jiang, M.; Koolmeister, C.; Wedell, A.; Taylor, R. W.; Wredenberg, A. et al.; Larsson, N.G.: FBXL4 deficiency increases mitochondrial removal by autophagy. EMBO Mol Med 12 (7), p. e11659 (2020)

Larsson, N.G.; Wedell, A.: Mitochondria in human disease. J Intern Med 287 (6), pp. 589 - 591 (2020)

Goebel, J.; Engelhardt, E.; Pelzer, P.; Sakthivelu, V.; Jahn, H. M.; Jevtic, M.; Folz-Donahue, K.; Kukat, C.; Schauss, A.; Frese, C. K. et al.; Giavalisco, P.; Ghanem, A.; Conzelmann, K. K.; Motori, E.; Bergami, M.: Mitochondria-Endoplasmic Reticulum Contacts in Reactive Astrocytes Promote Vascular Remodeling. Cell Metab 31 (4), pp. 791 - 808 e8 (2020)

Rumyantseva, A.; Motori, E.; Trifunovic, A.: DARS2 is indispensable for Purkinje cell survival and protects against cerebellar ataxia. Hum Mol Genet 29 (17), pp. 2845 - 2854 (2020)