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Treating mitochondrial disease caused by pathogenic mtDNA mutations

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Publications

Neuronal metabolic rewiring promotes resilience to neurodegeneration caused by mitochondrial dysfunction

Author(s): E. Motori, I. Atanassov, S. M. V. Kochan, K. Folz-Donahue, V. Sakthivelu, P. Giavalisco, N. Toni, J. Puyal, N.-G. Larsson
Published in: Science Advances, Issue 6/35, 2020, Page(s) eaba8271, ISSN 2375-2548
DOI: 10.1126/sciadv.aba8271

FBXL 4 deficiency increases mitochondrial removal by autophagy

Author(s): David Alsina, Oleksandr Lytovchenko, Aleksandra Schab, Ilian Atanassov, Florian A Schober, Min Jiang, Camilla Koolmeister, Anna Wedell, Robert W Taylor, Anna Wredenberg, Nils‐Göran Larsson
Published in: EMBO Molecular Medicine, Issue 12/7, 2020, ISSN 1757-4676
DOI: 10.15252/emmm.201911659

Small-molecule inhibitors of human mitochondrial DNA transcription

Author(s): Nina A. Bonekamp, Bradley Peter, Hauke S. Hillen, Andrea Felser, Tim Bergbrede, Axel Choidas, Moritz Horn, Anke Unger, Raffaella Di Lucrezia, Ilian Atanassov, Xinping Li, Uwe Koch, Sascha Menninger, Joanna Boros, Peter Habenberger, Patrick Giavalisco, Patrick Cramer, Martin S. Denzel, Peter Nussbaumer, Bert Klebl, Maria Falkenberg, Claes M. Gustafsson, Nils-Göran Larsson
Published in: Nature, Issue 588/7839, 2020, Page(s) 712-716, ISSN 0028-0836
DOI: 10.1038/s41586-020-03048-z

MitoRibo-Tag Mice Provide a Tool for In Vivo Studies of Mitoribosome Composition

Author(s): Jakob D. Busch, Miriam Cipullo, Ilian Atanassov, Ana Bratic, Eduardo Silva Ramos, Thomas Schöndorf, Xinping Li, Sarah F. Pearce, Dusanka Milenkovic, Joanna Rorbach, Nils-Göran Larsson
Published in: Cell Reports, Issue 29/6, 2019, Page(s) 1728-1738.e9, ISSN 2211-1247
DOI: 10.1016/j.celrep.2019.09.080

Mitochondrial fusion is required for regulation of mitochondrial DNA replication

Author(s): Eduardo Silva Ramos, Elisa Motori, Christian Brüser, Inge Kühl, Assa Yeroslaviz, Benedetta Ruzzenente, Johanna H. K. Kauppila, Jakob D. Busch, Kjell Hultenby, Bianca H. Habermann, Stefan Jakobs, Nils-Göran Larsson, Arnaud Mourier
Published in: PLOS Genetics, Issue 15/6, 2019, Page(s) e1008085, ISSN 1553-7404
DOI: 10.1371/journal.pgen.1008085

Modulation of mtDNA copy number ameliorates the pathological consequences of a heteroplasmic mtDNA mutation in the mouse

Author(s): R. Filograna, C. Koolmeister, M. Upadhyay, A. Pajak, P. Clemente, R. Wibom, M. L. Simard, A. Wredenberg, C. Freyer, J. B. Stewart, N. G. Larsson
Published in: Science Advances, Issue 5/4, 2019, Page(s) eaav9824, ISSN 2375-2548
DOI: 10.1126/sciadv.aav9824

Dinucleotide Degradation by REXO2 Maintains Promoter Specificity in Mammalian Mitochondria

Author(s): Thomas J. Nicholls, Henrik Spåhr, Shan Jiang, Stefan J. Siira, Camilla Koolmeister, Sushma Sharma, Johanna H.K. Kauppila, Min Jiang, Volkhard Kaever, Oliver Rackham, Andrei Chabes, Maria Falkenberg, Aleksandra Filipovska, Nils-Göran Larsson, Claes M. Gustafsson
Published in: Molecular Cell, Issue 76/5, 2019, Page(s) 784-796.e6, ISSN 1097-2765
DOI: 10.1016/j.molcel.2019.09.010