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1.

Replication and Transcription of Human Mitochondrial DNA.

Falkenberg, Maria; Larsson, Nils-Göran; Gustafsson, Claes M.

Annu Rev Biochem ; 2024 Apr 09.

Article in English | MEDLINE | ID: mdl-38594940

2.

Cell lineage-specific mitochondrial resilience during mammalian organogenesis.

Burr, Stephen P; Klimm, Florian; Glynos, Angelos; Prater, Malwina; Sendon, Pamella; Nash, Pavel; Powell, Christopher A; Simard, Marie-Lune; Bonekamp, Nina A; Charl, Julia; Diaz, Hector; Bozhilova, Lyuba V; Nie, Yu; Zhang, Haixin; Frison, Michele; Falkenberg, Maria; Jones, Nick; Minczuk, Michal; Stewart, James B; Chinnery, Patrick F.

Cell ; 186(6): 1212-1229.e21, 2023 03 16.

Article in English | MEDLINE | ID: mdl-36827974

3.

Non-coding 7S RNA inhibits transcription via mitochondrial RNA polymerase dimerization.

Zhu, Xuefeng; Xie, Xie; Das, Hrishikesh; Tan, Benedict G; Shi, Yonghong; Al-Behadili, Ali; Peter, Bradley; Motori, Elisa; Valenzuela, Sebastian; Posse, Viktor; Gustafsson, Claes M; Hällberg, B Martin; Falkenberg, Maria.

Cell ; 185(13): 2309-2323.e24, 2022 06 23.

Article in English | MEDLINE | ID: mdl-35662414

4.

Mechanisms and regulation of human mitochondrial transcription.

Tan, Benedict G; Gustafsson, Claes M; Falkenberg, Maria.

Nat Rev Mol Cell Biol ; 25(2): 119-132, 2024 Feb.

Article in English | MEDLINE | ID: mdl-37783784

5.

Maintenance and Expression of Mammalian Mitochondrial DNA.

Gustafsson, Claes M; Falkenberg, Maria; Larsson, Nils-Göran.

Annu Rev Biochem ; 85: 133-60, 2016 Jun 02.

Article in English | MEDLINE | ID: mdl-27023847

6.

The human mitochondrial genome contains a second light strand promoter.

Tan, Benedict G; Mutti, Christian D; Shi, Yonghong; Xie, Xie; Zhu, Xuefeng; Silva-Pinheiro, Pedro; Menger, Katja E; Díaz-Maldonado, Héctor; Wei, Wei; Nicholls, Thomas J; Chinnery, Patrick F; Minczuk, Michal; Falkenberg, Maria; Gustafsson, Claes M.

Mol Cell ; 82(19): 3646-3660.e9, 2022 10 06.

Article in English | MEDLINE | ID: mdl-36044900

7.

Dinucleotide Degradation by REXO2 Maintains Promoter Specificity in Mammalian Mitochondria.

Nicholls, Thomas J; Spåhr, Henrik; Jiang, Shan; Siira, Stefan J; Koolmeister, Camilla; Sharma, Sushma; Kauppila, Johanna H K; Jiang, Min; Kaever, Volkhard; Rackham, Oliver; Chabes, Andrei; Falkenberg, Maria; Filipovska, Aleksandra; Larsson, Nils-Göran; Gustafsson, Claes M.

Mol Cell ; 76(5): 784-796.e6, 2019 12 05.

Article in English | MEDLINE | ID: mdl-31588022

8.

Small-molecule inhibitors of human mitochondrial DNA transcription.

Bonekamp, Nina A; Peter, Bradley; Hillen, Hauke S; Felser, Andrea; Bergbrede, Tim; Choidas, Axel; Horn, Moritz; Unger, Anke; Di Lucrezia, Raffaella; Atanassov, Ilian; Li, Xinping; Koch, Uwe; Menninger, Sascha; Boros, Joanna; Habenberger, Peter; Giavalisco, Patrick; Cramer, Patrick; Denzel, Martin S; Nussbaumer, Peter; Klebl, Bert; Falkenberg, Maria; Gustafsson, Claes M; Larsson, Nils-Göran.

Nature ; 588(7839): 712-716, 2020 12.

Article in English | MEDLINE | ID: mdl-33328633

9.

Topoisomerase 3α Is Required for Decatenation and Segregation of Human mtDNA.

Nicholls, Thomas J; Nadalutti, Cristina A; Motori, Elisa; Sommerville, Ewen W; Gorman, Gráinne S; Basu, Swaraj; Hoberg, Emily; Turnbull, Doug M; Chinnery, Patrick F; Larsson, Nils-Göran; Larsson, Erik; Falkenberg, Maria; Taylor, Robert W; Griffith, Jack D; Gustafsson, Claes M.

Mol Cell ; 69(1): 9-23.e6, 2018 01 04.

Article in English | MEDLINE | ID: mdl-29290614

10.

The mutation R107Q alters mtSSB ssDNA compaction ability and binding dynamics.

Martucci, Martial; Moretton, Amandine; Tarrés-Solé, Aleix; Ropars, Virginie; Lambert, Louise; Vernet, Patrick; Solà, Maria; Falkenberg, Maria; Farge, Geraldine; van den Wildenberg, Siet.

Nucleic Acids Res ; 2024 May 14.

Article in English | MEDLINE | ID: mdl-38742632

11.

Structure casts light on mtDNA replication.

Falkenberg, Maria; Larsson, Nils-Göran.

Cell ; 139(2): 231-3, 2009 Oct 16.

Article in English | MEDLINE | ID: mdl-19837028

12.

Ribonucleotides embedded in template DNA impair mitochondrial RNA polymerase progression.

Singh, Meenakshi; Posse, Viktor; Peter, Bradley; Falkenberg, Maria; Gustafsson, Claes M.

Nucleic Acids Res ; 50(2): 989-999, 2022 01 25.

Article in English | MEDLINE | ID: mdl-35018464

13.

Mammalian RNase H1 directs RNA primer formation for mtDNA replication initiation and is also necessary for mtDNA replication completion.

Misic, Jelena; Milenkovic, Dusanka; Al-Behadili, Ali; Xie, Xie; Jiang, Min; Jiang, Shan; Filograna, Roberta; Koolmeister, Camilla; Siira, Stefan J; Jenninger, Louise; Filipovska, Aleksandra; Clausen, Anders R; Caporali, Leonardo; Valentino, Maria Lucia; La Morgia, Chiara; Carelli, Valerio; Nicholls, Thomas J; Wredenberg, Anna; Falkenberg, Maria; Larsson, Nils-Göran.

Nucleic Acids Res ; 50(15): 8749-8766, 2022 08 26.

Article in English | MEDLINE | ID: mdl-35947649

14.

Two type I topoisomerases maintain DNA topology in human mitochondria.

Menger, Katja E; Chapman, James; Díaz-Maldonado, Héctor; Khazeem, Mushtaq M; Deen, Dasha; Erdinc, Direnis; Casement, John W; Di Leo, Valeria; Pyle, Angela; Rodríguez-Luis, Alejandro; Cowell, Ian G; Falkenberg, Maria; Austin, Caroline A; Nicholls, Thomas J.

Nucleic Acids Res ; 50(19): 11154-11174, 2022 10 28.

Article in English | MEDLINE | ID: mdl-36215039

15.

DNA polymerase gamma mutations that impair holoenzyme stability cause catalytic subunit depletion.

Silva-Pinheiro, Pedro; Pardo-Hernández, Carlos; Reyes, Aurelio; Tilokani, Lisa; Mishra, Anup; Cerutti, Raffaele; Li, Shuaifeng; Rozsivalova, Dieu-Hien; Valenzuela, Sebastian; Dogan, Sukru A; Peter, Bradley; Fernández-Silva, Patricio; Trifunovic, Aleksandra; Prudent, Julien; Minczuk, Michal; Bindoff, Laurence; Macao, Bertil; Zeviani, Massimo; Falkenberg, Maria; Viscomi, Carlo.

Nucleic Acids Res ; 49(9): 5230-5248, 2021 05 21.

Article in English | MEDLINE | ID: mdl-33956154

16.

Accurate mapping of mitochondrial DNA deletions and duplications using deep sequencing.

Basu, Swaraj; Xie, Xie; Uhler, Jay P; Hedberg-Oldfors, Carola; Milenkovic, Dusanka; Baris, Olivier R; Kimoloi, Sammy; Matic, Stanka; Stewart, James B; Larsson, Nils-Göran; Wiesner, Rudolf J; Oldfors, Anders; Gustafsson, Claes M; Falkenberg, Maria; Larsson, Erik.

PLoS Genet ; 16(12): e1009242, 2020 12.

Article in English | MEDLINE | ID: mdl-33315859

17.

Mammalian mitochondrial DNA replication and mechanisms of deletion formation.

Falkenberg, Maria; Gustafsson, Claes M.

Crit Rev Biochem Mol Biol ; 55(6): 509-524, 2020 12.

Article in English | MEDLINE | ID: mdl-32972254

18.

TWNK in Parkinson's Disease: A Movement Disorder and Mitochondrial Disease Center Perspective Study.

Percetti, Marco; Franco, Giulia; Monfrini, Edoardo; Caporali, Leonardo; Minardi, Raffaella; La Morgia, Chiara; Valentino, Maria Lucia; Liguori, Rocco; Palmieri, Ilaria; Ottaviani, Donatella; Vizziello, Maria; Ronchi, Dario; Di Berardino, Federica; Cocco, Antoniangela; Macao, Bertil; Falkenberg, Maria; Comi, Giacomo Pietro; Albanese, Alberto; Giometto, Bruno; Valente, Enza Maria; Carelli, Valerio; Di Fonzo, Alessio.

Mov Disord ; 37(9): 1938-1943, 2022 09.

Article in English | MEDLINE | ID: mdl-35792653

19.

RNase H1 directs origin-specific initiation of DNA replication in human mitochondria.

Posse, Viktor; Al-Behadili, Ali; Uhler, Jay P; Clausen, Anders R; Reyes, Aurelio; Zeviani, Massimo; Falkenberg, Maria; Gustafsson, Claes M.

PLoS Genet ; 15(1): e1007781, 2019 01.

Article in English | MEDLINE | ID: mdl-30605451

20.

Second trimester fetal thymus size in association to preterm birth.

Porschen, Christian; Schmitz, Ralf; Schmidt, Rene; Oelmeier, Kathrin; Hammer, Kerstin; Falkenberg, Maria; Braun, Janina; Köster, Helen Ann; Steinhard, Johannes; Möllers, Mareike.

J Perinat Med ; 50(2): 144-149, 2022 Feb 23.

Article in English | MEDLINE | ID: mdl-34710316

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