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- Claes Gustafsson
Claes Gustafsson
Professor
Avd för medicinsk kemi och cellbiologiProprefekt
Institutionen för biomedicinOm Claes Gustafsson
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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
Fler publikationer
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