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Treating mitochondrial disease caused by pathogenic mtDNA mutations

Pubblicazioni

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

Autori: Mara Mennuni; Roberta Filograna; Andrea Felser; Andrea Felser; Nina A Bonekamp; Nina A Bonekamp; Patrick Giavalisco; Oleksandr Lytovchenko; Nils-Göran Larsson
Pubblicato in: EMBO Reports, 2022, ISSN 1469-3178
Editore: 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.

Autori: 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
Pubblicato in: Sci Adv, 2021, ISSN 2375-2548
Editore: AAAS
DOI: 10.1126/sciadv.abf8631

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

Autori: Roberta Filograna; Roberta Filograna; Mara Mennuni; Mara Mennuni; David Alsina; David Alsina; Nils-Göran Larsson; Nils-Göran Larsson
Pubblicato in: Febs Letters, 2020, ISSN 1873-3468
Editore: 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

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

Neuronal metabolic rewiring promotes resilience to neurodegeneration caused by mitochondrial dysfunction

Autori: E. Motori, I. Atanassov, S. M. V. Kochan, K. Folz-Donahue, V. Sakthivelu, P. Giavalisco, N. Toni, J. Puyal, N.-G. Larsson
Pubblicato in: Science Advances, Numero 6/35, 2020, Pagina/e eaba8271, ISSN 2375-2548
Editore: Science Advances
DOI: 10.1126/sciadv.aba8271

FBXL 4 deficiency increases mitochondrial removal by autophagy

Autori: 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
Pubblicato in: EMBO Molecular Medicine, Numero 12/7, 2020, ISSN 1757-4676
Editore: John Wiley & Sons Ltd.
DOI: 10.15252/emmm.201911659

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

Autori: 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
Pubblicato in: Life Sci Alliance, 2021, ISSN 2575-1077
Editore: Rockefeller University Press
DOI: 10.26508/lsa.202101034

Small-molecule inhibitors of human mitochondrial DNA transcription

Autori: 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
Pubblicato in: Nature, Numero 588/7839, 2020, Pagina/e 712-716, ISSN 0028-0836
Editore: Nature Publishing Group
DOI: 10.1038/s41586-020-03048-z

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

Autori: 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
Pubblicato in: Cell Reports, Numero 29/6, 2019, Pagina/e 1728-1738.e9, ISSN 2211-1247
Editore: Cell Press
DOI: 10.1016/j.celrep.2019.09.080

Mitochondrial fusion is required for regulation of mitochondrial DNA replication

Autori: 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
Pubblicato in: PLOS Genetics, Numero 15/6, 2019, Pagina/e e1008085, ISSN 1553-7404
Editore: Public Library of Science
DOI: 10.1371/journal.pgen.1008085

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

Autori: 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
Pubblicato in: PLoS Genetics, 2022, ISSN 1553-7390
Editore: Public Library of Science
DOI: 10.1371/journal.pgen.1010190

Dinucleotide Degradation by REXO2 Maintains Promoter Specificity in Mammalian Mitochondria

Autori: 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
Pubblicato in: Molecular Cell, Numero 76/5, 2019, Pagina/e 784-796.e6, ISSN 1097-2765
Editore: Cell Press
DOI: 10.1016/j.molcel.2019.09.010

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

Autori: Laura S Kremer, Lyuba V Bozhilova, Diana Rubalcava-Gracia, Roberta Filograna, Mamta Upadhyay, Camilla Koolmeister, Patrick F Chinnery, Nils-Göran Larsson
Pubblicato in: PLOS Genetics, 2023, ISSN 1553-7390
Editore: Public Library of Science

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