Treating mitochondrial disease caused by pathogenic mtDNA mutations

Publications

Metabolic resistance to the inhibition of mitochondrial transcription revealed by CRISPR-Cas9 screen.

Author(s): Mara Mennuni; Roberta Filograna; Andrea Felser; Andrea Felser; Nina A Bonekamp; Nina A Bonekamp; Patrick Giavalisco; Oleksandr Lytovchenko; Nils-Göran Larsson
Published in: EMBO Reports, 2022, ISSN 1469-3178
Publisher: European Molecular Biology Organization, Nature Portfolio
DOI: 10.15252/embr.202153054

The mitochondrial single-stranded DNA binding protein is essential for initiation of mtDNA replication.

Author(s): Min Jiang; Min Jiang; Xie Xie; Xuefeng Zhu; Shan Jiang; Dusanka Milenkovic; Jelena Misic; Yonghong Shi; Nirwan Tandukar; Xinping Li; Ilian Atanassov; Louise Jenninger; Emily Hoberg; Sara Albarran-Gutierrez; Zsolt Szilagyi; Bertil Macao; Stefan J. Siira; Stefan J. Siira; Valerio Carelli; Jack D. Griffith; Claes M. Gustafsson; Thomas J. Nicholls; Aleksandra Filipovska; Aleksandra Filipovska; Nils-G
Published in: Sci Adv, 2021, ISSN 2375-2548
Publisher: AAAS
DOI: 10.1126/sciadv.abf8631

Mitochondrial DNA copy number in human disease: the more the better?

Author(s): Roberta Filograna; Roberta Filograna; Mara Mennuni; Mara Mennuni; David Alsina; David Alsina; Nils-Göran Larsson; Nils-Göran Larsson
Published in: Febs Letters, 2020, ISSN 1873-3468
Publisher: Febs Letters
DOI: 10.1002/1873-3468.14021

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

Author(s): Filograna Roberta, Koolmeister Coolmeister, Upadhyay Mamta, Pajak A, Clemente Paula, Wibom Rolf, Simard ML, Wredenberg Anna, Freyer Chris, Stewart Jim B, Larsson Nils-Göran
Published in: Science Advances, 2019, ISSN 2375-2548
Publisher: AAAS
DOI: 10.1126/sciadv.aav9824

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
Publisher: Science Advances
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
Publisher: John Wiley & Sons Ltd.
DOI: 10.15252/emmm.201911659

High levels of TFAM repress mammalian mitochondrial DNA transcription in vivo.

Author(s): Nina A. Bonekamp; Min Jiang; Min Jiang; Elisa Motori; Elisa Motori; Rodolfo Garcia Villegas; Camilla Koolmeister; Ilian Atanassov; Andrea Mesaros; Chan Bae Park; Nils-Göran Larsson; Nils-Göran Larsson
Published in: Life Sci Alliance, 2021, ISSN 2575-1077
Publisher: Rockefeller University Press
DOI: 10.26508/lsa.202101034

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
Publisher: Nature Publishing Group
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
Publisher: Cell Press
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
Publisher: Public Library of Science
DOI: 10.1371/journal.pgen.1008085

Mice lacking the mitochondrial exonuclease MGME1 develop inflammatory kidney disease with glomerular dysfunction

Author(s): Dusanka Milenkovic,Adrián Sanz-Moreno,Julia Calzada-Wack,Birgit Rathkolb,Oana Veronica Amarie,Raffaele Gerlini,Antonio Aguilar-Pimentel,Jelena Misic,Marie-Lune Simard,Eckhard Wolf,Helmut Fuchs,Valerie Gailus-Durner,Martin Hrabě de Angelis ,Nils-Göran Larsson
Published in: PLoS Genetics, 2022, ISSN 1553-7390
Publisher: Public Library of Science
DOI: 10.1371/journal.pgen.1010190

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
Publisher: Cell Press
DOI: 10.1016/j.molcel.2019.09.010

A role for BCL2L13 and autophagy in germline purifying selection of mtDNA

Author(s): Laura S Kremer, Lyuba V Bozhilova, Diana Rubalcava-Gracia, Roberta Filograna, Mamta Upadhyay, Camilla Koolmeister, Patrick F Chinnery, Nils-Göran Larsson
Published in: PLOS Genetics, 2023, ISSN 1553-7390
Publisher: Public Library of Science

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