Claes Gustafsson

• Modelling POLG mutations in mice unravels a critical role of POLγΒ in regulating phenotypic severity

  Samantha Corra, Alessandro Zuppardo, Sebastian Valenzuela, Louise Jenninger, Raffaele Cerutti, Sirelin Sillamaa, Emily Hoberg, Katarina Johansson, Urska Rovsnik, Sara Volta, Pedro Silva-Pinheiro, Hannah Davis, Aleksandra Trifunovic, Michal Minczuk, Claes M Gustafsson, Anu Suomalainen, Massimo Zeviani, Bertil Macao, Xuefeng Zhu, Maria Falkenberg, Carlo Viscomi
  NATURE COMMUNICATIONS - 2025
• Small molecules restore mutant mitochondrial DNA polymerase activity

  Sebastian Valenzuela, Xuefeng Zhu, Bertil Macao, Mattias Stamgren, Carol Geukens, Paul S. Charifson, Gunther Kern, Emily Hoberg, Louise Jenninger, Anja V. Gruszczyk, Seoeun Lee, Katarina Johansson, Javier Miralles Fuste, Yonghong Shi, S. Jordan Kerns, Laleh Arabanian, Gabriel Martinez Botella, Sofie Ekstroem, Jeremy Green, Andrew M. Griffin, Carlos Pardo-Hernandez, Thomas A. Keating, Barbara Kuppers-Munther, Nils-Goran Larsson, Cindy Phan, Viktor Posse, Juli E. Jones, Xie Xie, Simon Giroux, Claes M Gustafsson, Maria Falkenberg
  NATURE - 2025
• Replication and Transcription of Human Mitochondrial DNA

  Maria Falkenberg, Nils-Goran Larsson, Claes M Gustafsson
  ANNUAL REVIEW OF BIOCHEMISTRY - 2024
• Mechanisms and regulation of human mitochondrial transcription

  B. G. Tan, Claes M Gustafsson, Maria Falkenberg
  Nature Reviews Molecular Cell Biology - 2024
• TEFM variants impair mitochondrial transcription causing childhood-onset neurological disease

  L. Van Haute, E. O'Connor, Hector Diaz-Maldonado, B. Munro, K. Polavarapu, D. H. Hock, G. Arunachal, A. Athanasiou-Fragkouli, M. Bardhan, M. Barth, D. Bonneau, N. Brunetti-Pierri, G. Cappuccio, N. J. Caruana, N. Dominik, H. Goel, G. Helman, H. Houlden, G. Lenaers, K. Mention, D. Murphy, B. Nandeesh, C. Olimpio, C. A. Powell, V. Preethish-Kumar, V. Procaccio, R. Rius, P. Rebelo-Guiomar, C. Simons, S. Vengalil, M. S. Zaki, A. Ziegler, D. R. Thorburn, D. A. Stroud, R. Maroofian, J. Christodoulou, Claes M Gustafsson, A. Nalini, H. Lochmuller, M. Minczuk, R. Horvath
  Nature Communications - 2023
• Pathological variants in TOP3A cause distinct disorders of mitochondrial and nuclear genome stability

  Direnis Erdinc, A. Rodriguez-Luis, M. R. Fassad, S. Mackenzie, C. M. Watson, Sebastian Valenzuela, Xie Xie, K. E. Menger, K. Sergeant, K. Craig, S. Hopton, G. Falkous, J. Poulton, H. Garcia-Moreno, P. Giunti, C. A. D. Aschoff, J. A. M. Saute, A. J. Kirby, C. Toro, L. Wolfe, D. Novacic, L. Greenbaum, A. Eliyahu, O. Barel, Y. Anikster, R. McFarland, G. S. Gorman, A. M. Schaefer, Claes M Gustafsson, R. W. Taylor, Maria Falkenberg, T. J. Nicholls
  Embo Molecular Medicine - 2023
• Lsm7 phase-separated condensates trigger stress granule formation

  Michelle Lindström, Lihua Chen, Shan Jiang, Dan Zhang, Yuan Gao, J. Zheng, Xinxin Hao, Xiaoxue Yang, Arpitha Kabbinale, Johannes Thoma, Lisa Metzger, D. Y. Zhang, Xuefeng Zhu, H. S. Liu, Claes M Gustafsson, Björn Marcus Burmann, J. Winderickx, Per Sunnerhagen, Beidong Liu
  Nature Communications - 2022
• Article Non-coding 7S RNA inhibits transcription via mitochondrial RNA polymerase dimerization

  Xuefeng Zhu, Xie Xie, H. Das, Benedict Tan, Yonghong Shi, Ali Al-Behadili, Bradley Peter, E. Motori, Sebastian Valenzuela, Viktor Posse, Claes M Gustafsson, B. M. Hallberg, Maria Falkenberg
  Cell - 2022
• Ribonucleotides embedded in template DNA impair mitochondrial RNA polymerase progression

  Meenakshi Singh, Viktor Posse, Bradley Peter, Maria Falkenberg, Claes M Gustafsson
  Nucleic Acids Research - 2022
• The human mitochondrial genome contains a second light strand promoter

  Benedict Tan, C. D. Mutti, Yonghong Shi, Xie Xie, Xuefeng Zhu, P. Silva-Pinheiro, K. E. Menger, Hector Diaz-Maldonado, W. Wei, T. J. Nicholls, P. F. Chinnery, M. Minczuk, Maria Falkenberg, Claes M Gustafsson
  Molecular Cell - 2022
• The mitochondrial single-stranded DNA binding protein is essential for initiation of mtDNA replication

  M. Jiang, Xie Xie, Xuefeng Zhu, S. Jiang, D. Milenkovic, J. Misic, Yonghong Shi, N. Tandukar, X. Li, I. Atanassov, Louise Jenninger, Emily Hoberg, S. Albarran-Gutierrez, Zsolt Szilagyi, Bertil Macao, S. J. Siira, V. Carelli, J. D. Griffith, Claes M Gustafsson, T. J. Nicholls, A. Filipovska, N. G. Larsson, Maria Falkenberg
  Science Advances - 2021
• POLRMT mutations impair mitochondrial transcription causing neurological disease

  M. Olahova, Bradley Peter, Zsolt Szilagyi, Hector Diaz-Maldonado, Meenakshi Singh, E. W. Sommerville, E. L. Blakely, J. J. Collier, Emily Hoberg, V. Stranecky, H. Hartmannova, A. J. Bleyer, K. L. McBride, S. A. Bowden, Z. Korandova, A. Pecinova, H. H. Ropers, K. Kahrizi, H. Najmabadi, M. A. Tarnopolsky, L. I. Brady, K. N. Weaver, C. E. Prada, K. Ounap, M. H. Wojcik, S. Pajusalu, S. B. Syeda, L. Pais, E. A. Estrella, C. C. Bruels, L. M. Kunkel, P. B. Kang, P. E. Bonnen, T. Mracek, S. Kmoch, G. S. Gorman, Maria Falkenberg, Claes M Gustafsson, R. W. Taylor
  Nature Communications - 2021
• Mammalian mitochondrial DNA replication and mechanisms of deletion formation

  Maria Falkenberg, Claes M Gustafsson
  Critical Reviews in Biochemistry and Molecular Biology - 2020
• Absolute yeast mitochondrial proteome quantification reveals trade-off between biosynthesis and energy generation during diauxic shift

  Francesca Di Bartolomeo, C. Malina, K. Campbell, M. Mormino, Johannes Fuchs, Egor Vorontsov, Claes M Gustafsson, J. Nielsen
  Proceedings of the National Academy of Sciences of the United States of America - 2020
• Small-molecule inhibitors of human mitochondrial DNA transcription

  N. A. Bonekamp, Bradley Peter, H. S. Hillen, A. Felser, T. Bergbrede, A. Choidas, M. Horn, A. Unger, R. Di Lucrezia, I. Atanassov, X. P. Li, U. Koch, S. Menninger, J. Boros, P. Habenberger, P. Giavalisco, P. Cramer, M. S. Denzel, P. Nussbaumer, B. Klebl, Maria Falkenberg, Claes M Gustafsson, N. G. Larsson
  Nature - 2020
• Accurate mapping of mitochondrial DNA deletions and duplications using deep sequencing

  Swaraj Basu, Xie Xie, Jay (Jennifer) Uhler, Carola Oldfors Hedberg, D. Milenkovic, O. R. Baris, S. Kimoloi, S. Matic, J. B. Stewart, N. G. Larsson, R. J. Wiesner, Anders Oldfors, Claes M Gustafsson, Maria Falkenberg, Erik Larsson
  PLoS Genetics - 2020
• Yeast mismatch repair components are required for stable inheritance of gene silencing

  Qian Liu, Xuefeng Zhu, Michelle Lindström, Yonghong Shi, Ju Zheng, Xinxin Hao, Claes M Gustafsson, Beidong Liu
  Plos Genetics - 2020
• TEFM regulates both transcription elongation and RNA processing in mitochondria

  Shan Jiang, Camilla Koolmeister, Jelena Misic, Stefan Siira, Inge Kuehl, Eduardo Silva Ramos, Maria Miranda, Min Jiang, Viktor Posse, Oleksandr Lytovchenko, Ilian Atanassov, Florian A. Schober, Rolf Wibom, Kjell Hultenby, Dusanka Milenkovic, Claes M Gustafsson, Aleksandra Filipovska, Nils-Goeran Larsson
  EMBO REPORTS - 2019
• Dinucleotide Degradation by REXO2 Maintains Promoter Specificity in Mammalian Mitochondria

  Thomas J. Nicholls, H. Spahr, S. Jiang, S. J. Siira, C. Koolmeister, S. Sharma, J. H. K. Kauppila, M. Jiang, V. Kaever, O. Rackham, A. Chabes, Maria Falkenberg, A. Filipovska, N. G. Larsson, Claes M Gustafsson
  Molecular Cell - 2019
• Copy-choice recombination during mitochondrial L-strand synthesis causes DNA deletions

  Örjan Persson, Yazh Muthukumar, Swaraj Basu, Louise Jenninger, Jay (Jennifer) Uhler, Anna-Karin Berglund, R. McFarland, R. W. Taylor, Claes M Gustafsson, Erik Larsson, Maria Falkenberg
  Nature Communications - 2019
• RNase H1 directs origin-specific initiation of DNA replication in human mitochondria

  Viktor Posse, Ali Al-Behadili, Jay (Jennifer) Uhler, Anders R Clausen, A. Reyes, M. Zeviani, Maria Falkenberg, Claes M Gustafsson
  Plos Genetics - 2019
• Separating and Segregating the Human Mitochondrial Genome

  Thomas J. Nicholls, Claes M Gustafsson
  Trends in Biochemical Sciences - 2018
• Topoisomerase 3α Is Required for Decatenation and Segregation of Human mtDNA

  Thomas J. Nicholls, C. A. Nadalutti, E. Motori, E. W. Sommerville, G. S. Gorman, Swaraj Basu, Emily Hoberg, D. M. Turnbull, P. F. Chinnery, N. G. Larsson, Erik Larsson, Maria Falkenberg, R. W. Taylor, J. D. Griffith, Claes M Gustafsson
  Molecular Cell - 2018
• An Adaptable High-Throughput Technology Enabling the Identification of Specific Transcription Modulators

  T. Bergbrede, Emily Hoberg, N. G. Larsson, Maria Falkenberg, Claes M Gustafsson
  Slas Discovery - 2017
• Nucleotide pools dictate the identity and frequency of ribonucleotide incorporation in mitochondrial DNA.

  Anna-Karin Berglund, Clara Navarrete, Martin K M Engqvist, Emily Hoberg, Zsolt Szilagyi, Robert W Taylor, Claes M Gustafsson, Maria Falkenberg, Anders R Clausen
  PLoS genetics - 2017
• Human Mitochondrial Transcription Factor B2 Is Required for Promoter Melting during Initiation of Transcription

  Viktor Posse, Claes M Gustafsson
  Journal of Biological Chemistry - 2017
• Cyclin C influences the timing of mitosis in fission yeast.

  Gabor Banyai, Zsolt Szilagyi, Vera Baraznenok, Olga Khorosjutina, Claes M Gustafsson
  Molecular biology of the cell - 2017
• Mutations in mitochondrial DNA causing tubulointerstitial kidney disease

  T. M. Connor, S. Hoer, A. Mallett, D. P. Gale, A. Gomez-Duran, Viktor Posse, R. Antrobus, P. Moreno, M. Sciacovelli, C. Frezza, J. Duff, N. S. Sheerin, J. A. Sayer, M. Ashcroft, M. S. Wiesener, G. Hudson, Claes M Gustafsson, P. F. Chinnery, P. H. Maxwell
  Plos Genetics - 2017
• POLRMT regulates the switch between replication primer formation and gene expression of mammalian mtDNA

  I. Kuhl, M. Miranda, Viktor Posse, D. Milenkovic, A. Mourier, S. J. Siira, N. A. Bonekamp, U. Neumann, A. Filipovska, P. L. Polosa, Claes M Gustafsson, N. G. Larsson
  Science Advances - 2016
• MGME1 processes flaps into ligatable nicks in concert with DNA polymerase gamma during mtDNA replication

  Jay (Jennifer) Uhler, Christian Thörn, Thomas J. Nicholls, S. Matic, D. Milenkovic, Claes M Gustafsson, Maria Falkenberg
  Nucleic Acids Research - 2016
• Mitochondrial transcription termination factor 1 directs polar replication fork pausing

  Yonghong Shi, Viktor Posse, Xuefeng Zhu, A. K. Hyvarinen, H. T. Jacobs, Maria Falkenberg, Claes M Gustafsson
  Nucleic Acids Research - 2016
• Maintenance and Expression of Mammalian Mitochondrial DNA

  Claes M Gustafsson, Maria Falkenberg, N. G. Larsson
  Annual Review of Biochemistry - 2016
• Loss of the Mediator subunit Med20 affects transcription of tRNA and other non-coding RNA genes in fission yeast

  Jonas O P Carlsten, Xuefeng Zhu, Marcela Davila Lopez, Tore Samuelsson, Claes M Gustafsson
  Biochimica Et Biophysica Acta-Gene Regulatory Mechanisms - 2016
• Simultaneous DNA and RNA Mapping of Somatic Mitochondrial Mutations across Diverse Human Cancers.

  James B Stewart, Babak Alaei-Mahabadi, Radhakrishnan Sabarinathan, Tore Samuelsson, Jan Gorodkin, Claes M Gustafsson, Erik Larsson
  PLoS genetics - 2015
• Regulation of DNA replication at the end of the mitochondrial D-loop involves the helicase TWINKLE and a conserved sequence element

  Elisabeth Jemt, Örjan Persson, Yonghong Shi, Majda Mehmedovic, Jay (Jennifer) Uhler, Marcela Davila Lopez, C. Freyer, Claes M Gustafsson, Tore Samuelsson, Maria Falkenberg
  Nucleic Acids Research - 2015
• MEG3 long noncoding RNA regulates the TGF-β pathway genes through formation of RNA–DNA triplex structures

  Tanmoy Mondal, Santhilal Subhash, Roshan Vaid, Stefan Enroth, Sireesha Uday, Björn Reinius, Sanhita Mitra, Arif Mohammed, Alva Rani James, Emily Hoberg, Aristidis Moustakas, Ulf Gyllensten, Steven J.M. Jones, Claes M Gustafsson, Andrew H Sims, Fredrik Westerlund, Eduardo Gorab, Chandrasekhar Kanduri
  Nature Communications - 2015
• Mediator tail subunits can form amyloid-like aggregates in vivo and affect stress response in yeast

  Xuefeng Zhu, Lihua Chen, Jonas O P Carlsten, Qian Liu, Junsheng Yang, Beidong Liu, Claes M Gustafsson
  Nucleic Acids Research - 2015
• The exonuclease activity of DNA polymerase gamma is required for ligation during mitochondrial DNA replication

  Bertil Macao, Jay (Jennifer) Uhler, Triinu Siibak, Xuefeng Zhu, Yonghong Shi, Wenwen Sheng, Monica Olsson, J. B. Stewart, Claes M Gustafsson, Maria Falkenberg
  Nature Communications - 2015
• TEFM is a potent stimulator of mitochondrial transcription elongation in vitro

  Viktor Posse, S. Shahzad, Maria Falkenberg, B. M. Hallberg, Claes M Gustafsson
  Nucleic Acids Research - 2015
• Whole exome sequencing reveals mutations in NARS2 and PARS2, encoding the mitochondrial asparaginyl-tRNA synthetase and prolyl-tRNA synthetase, in patients with Alpers syndrome.

  Kalliopi Sofou, Gittan Kollberg, Maria Holmström, Marcela Davila Lopez, Niklas Darin, Claes M Gustafsson, Elisabeth Holme, Anders Oldfors, Mar Tulinius, Jorge Asin-Cayuela
  Molecular genetics & genomic medicine - 2015
• In Vivo Occupancy of Mitochondrial Single-Stranded DNA Binding Protein Supports the Strand Displacement Mode of DNA Replication

  Javier Miralles Fusté, Yonghong Shi, Sjoerd Wanrooij, Xuefeng Zhu, Elisabeth Jemt, Örjan Persson, N. Sabouri, Claes M Gustafsson, Maria Falkenberg
  Plos Genetics - 2014
• The amino terminal extension of mammalian mitochondrial RNA polymerase ensures promoter specific transcription initiation

  Viktor Posse, Emily Hoberg, Anke Dierckx, Saba Shahzad, Camilla Koolmeister, Nils-Göran Larsson, Marcus Wilhelmsson, B. Martin Hällberg, Claes M Gustafsson
  Nucleic Acids Research - 2014
• Essential Genetic Interactors of SIR2 Required for Spatial Sequestration and Asymmetrical Inheritance of Protein Aggregates

  J. Song, Q. Yang, Junsheng Yang, Lisa C Larsson, Xinxin Hao, Xuefeng Zhu, Sandra Malmgren Hill, Marija Cvijovic, Julia Fernandez-Rodriguez, Julie Grantham, Claes M Gustafsson, Beidong Liu, Thomas Nyström
  PLoS Genetics - 2014
• Mediator Can Regulate Mitotic Entry and Direct Periodic Transcription in Fission Yeast

  Gabor Banyai, Marcela Davila Lopez, Zsolt Szilagyi, Claes M Gustafsson
  Molecular and Cellular Biology - 2014
• In Vitro-Reconstituted Nucleoids Can Block Mitochondrial DNA Replication and Transcription

  G. Farge, Majda Mehmedovic, M. Baclayon, Smjl van den Wildenberg, W. H. Roos, Claes M Gustafsson, G. J. L. Wuite, Maria Falkenberg
  Cell Reports - 2014
• The UbL protein UBTD1 stably interacts with the UBE2D family of E2 ubiquitin conjugating enzymes

  Jay (Jennifer) Uhler, H. Spahr, G. Farge, S. Clavel, N. G. Larsson, Maria Falkenberg, Tore Samuelsson, Claes M Gustafsson
  Biochemical and Biophysical Research Communications - 2014
• Hereditary myopathy with early respiratory failure is associated with misfolding of the titin fibronectin III 119 subdomain

  Carola Hedberg, Alejandro Gomez Toledo, Claes M Gustafsson, Göran Larson, Anders Oldfors, Bertil Macao
  Neuromuscular Disorders - 2014
• MTERF1 Binds mtDNA to Prevent Transcriptional Interference at the Light-Strand Promoter but Is Dispensable for rRNA Gene Transcription Regulation

  M. Terzioglu, B. Ruzzenente, J. Harmel, A. Mourier, Elisabeth Jemt, Marcela Davila Lopez, C. Kukat, J. B. Stewart, R. Wibom, C. Meharg, B. Habermann, Maria Falkenberg, Claes M Gustafsson, C. B. Park, N. G. Larsson
  Cell Metabolism - 2013
• The multitalented Mediator complex

  Jonas O P Carlsten, Xuefeng Zhu, Claes M Gustafsson
  Trends in Biochemical Sciences - 2013
• Emerging roles of Cdk8 in cell cycle control

  Zsolt Szilagyi, Claes M Gustafsson
  Biochimica Et Biophysica Acta-Gene Regulatory Mechanisms - 2013

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