- Hem
- Om universitetet
- Hitta person
- Maria Falkenberg
Maria Falkenberg
Professor
Avd för medicinsk kemi och cellbiologi-
The mutation R107Q alters mtSSB ssDNA compaction ability and binding
dynamics
Martial Martucci, Amandine Moretton, Aleix Tarres-Sole, Virginie Ropars, Louise Lambert, Patrick Vernet, Maria Sola, Maria Falkenberg, Geraldine Farge, Siet van den Wildenberg
NUCLEIC ACIDS RESEARCH - 2024 -
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 -
The disease-causing mutation p.F907I reveals a novel pathogenic mechanism for POL?-related
diseases
Direnis Erdinc, Bertil Macao, Sebastian Valenzuela, N. Lesko, K. Naess, Bradley Peter, H. Bruhn, A. Wedell, A. Wredenberg, Maria Falkenberg
Biochimica Et Biophysica Acta-Molecular Basis of Disease - 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 -
Cell lineage-specific mitochondrial resilience during mammalian
organogenesis
S. P. Burr, F. Klimm, A. Glynos, M. Prater, P. Sendon, P. Nash, C. A. Powell, M. L. Simard, N. A. Bonekamp, J. Charl, Hector Diaz-Maldonado, L. V. Bozhilova, Y. Nie, H. X. Zhang, M. Frison, Maria Falkenberg, N. Jones, M. Minczuk, J. B. Stewart, P. F. Chinnery
Cell - 2023 -
In Vitro Assays of TWINKLE
Function.
Jay P Uhler, Ulrika Alexandersson, Maria Falkenberg
Methods in molecular biology (Clifton, N.J.) - 2023 -
Disease causing mutation (P178L) in mitochondrial transcription factor A results in impaired mitochondrial transcription
initiation
Majda Mehmedovic, M. Martucci, H. Spahr, Layal Ishak, Anup Mishra, Maria E. Sánchez-Sandoval, Carlos Pardo-Hernandez, Bradley Peter, S. M. van den Wildenberg, Maria Falkenberg, G. Farge
Biochimica Et Biophysica Acta-Molecular Basis of Disease - 2022 -
Two type I topoisomerases maintain DNA topology in human
mitochondria
K. E. Menger, J. Chapman, Hector Diaz-Maldonado, M. M. Khazeem, D. Deen, Direnis Erdinc, J. W. Casement, V. Di Leo, A. Pyle, A. Rodriguez-Luis, I. G. Cowell, Maria Falkenberg, C. A. Austin, T. J. Nicholls
Nucleic Acids Research - 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 -
TWNK in Parkinson's Disease: A Movement Disorder and Mitochondrial Disease Center Perspective
Study
M. Percetti, G. Franco, E. Monfrini, L. Caporali, R. Minardi, C. La Morgia, M. L. Valentino, R. Liguori, I. Palmieri, D. Ottaviani, M. Vizziello, D. Ronchi, F. Di Berardino, A. Cocco, Bertil Macao, Maria Falkenberg, G. P. Comi, A. Albanese, B. Giometto, E. M. Valente, V. Carelli, A. Di Fonzo
Movement Disorders - 2022 -
Mammalian RNase H1 directs RNA primer formation for mtDNA replication initiation and is also necessary for mtDNA replication
completion
J. Misic, D. Milenkovic, Ali Al-Behadili, Xie Xie, M. Jiang, S. Jiang, R. Filograna, C. Koolmeister, S. J. Siira, Louise Jenninger, A. Filipovska, Anders R Clausen, L. Caporali, M. L. Valentino, C. La Morgia, V. Carelli, T. J. Nicholls, A. Wredenberg, Maria Falkenberg, N. G. Larsson
Nucleic Acids Research - 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 -
DNA polymerase gamma mutations that impair holoenzyme stability cause catalytic subunit
depletion
P. Silva-Pinheiro, Carlos Pardo-Hernandez, A. Reyes, L. Tilokani, Anup Mishra, R. Cerutti, S. F. Li, D. H. Rozsivalova, Sebastian Valenzuela, S. A. Dogan, Bradley Peter, P. Fernandez-Silva, A. Trifunovic, J. Prudent, M. Minczuk, L. Bindoff, Bertil Macao, M. Zeviani, Maria Falkenberg, C. Viscomi
Nucleic Acids Research - 2021 -
Functional analysis of a novel POL gamma A mutation associated with a severe perinatal mitochondrial
encephalomyopathy
Niklas Darin, Triinu Siibak, Bradley Peter, Carola Oldfors Hedberg, Gittan Kollberg, Vassili Kalbin, Ali-Reza Moslemi, Bertil Macao, Anders Oldfors, Maria Falkenberg
Neuromuscular Disorders - 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 -
Mitochondrial DNA variants in inclusion body myositis characterized by deep
sequencing
Carola Oldfors Hedberg, Ulrika Lindgren, Swaraj Basu, Kittichate Visuttijai, Christopher Lindberg, Maria Falkenberg, Erik Larsson Lekholm, Anders Oldfors
Brain Pathology - 2021 -
In Vitro Analysis of mtDNA
Replication
Jay (Jennifer) Uhler, Maria Falkenberg
Mitochondrial Gene Expression - 2021 -
Deep sequencing of mitochondrial DNA and characterization of a novel POLG mutation in a patient with
arPEO.
Carola Oldfors Hedberg, Bertil Macao, Swaraj Basu, Christopher Lindberg, Bradley Peter, Jay (Jennifer) Uhler, Erik Larsson, Maria Falkenberg, Anders Oldfors
Neurology. Genetics - 2020 -
SSBP1 mutations cause mtDNA depletion underlying a complex optic atrophy
disorder
V. Del Dotto, F. Ullah, I. Di Meo, P. Magini, M. Gusic, A. Maresca, L. Caporali, F. Palombo, F. Tagliavini, E. H. Baugh, Bertil Macao, Zsolt Szilagyi, C. Peron, M. A. Gustafson, K. Khan, C. La Morgia, P. Barboni, M. Carbonelli, M. L. Valentino, R. Liguori, V. Shashi, J. Sullivan, S. Nagaraj, M. El-Dairi, A. Iannaccone, I. Cutcutache, E. Bertini, R. Carrozzo, F. Emma, F. Diomedi-Camassei, C. Zanna, M. Armstrong, M. Page, N. Stong, S. Boesch, R. Kopajtich, S. Wortmann, W. Sperl, E. E. Davis, W. C. Copeland, M. Seri, Maria Falkenberg, H. Prokisch, N. Katsanis, V. Tiranti, T. Pippucci, V. Carelli
Journal of Clinical Investigation - 2020 -
Mammalian mitochondrial DNA replication and mechanisms of deletion
formation
Maria Falkenberg, Claes M Gustafsson
Critical Reviews in Biochemistry and Molecular Biology - 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 -
TWINKLE and Other Human Mitochondrial DNA Helicases: Structure, Function and
Disease.
Bradley Peter, Maria Falkenberg
Genes - 2020 -
Corrigendum to: Structural basis for adPEO-causing mutations in the mitochondrial TWINKLE
helicase
Bradley Peter, G. Farge, Carlos Pardo-Hernandez, Stefan Tångefjord, Maria Falkenberg
Human molecular genetics - 2020 -
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 -
Organization of DNA in mammalian
mitochondria
G. É R. Farge, Maria Falkenberg
International Journal of Molecular Sciences - 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 -
Structural basis for adPEO-causing mutations in the mitochondrial TWINKLE
helicase.
Bradley Peter, Geraldine Farge, Carlos Pardo-Hernandez, Stefan Tångefjord, Maria Falkenberg
Human molecular genetics - 2019 -
Mice lacking the mitochondrial exonuclease MGME1 accumulate mtDNA deletions without developing
progeria
S. Matic, M. Jiang, Thomas J. Nicholls, Jay (Jennifer) Uhler, C. Dirksen-Schwanenland, P. L. Polosa, M. L. Simard, X. P. Li, I. Atanassov, O. Rackham, A. Filipovska, J. B. Stewart, Maria Falkenberg, N. G. Larsson, D. Milenkovic
Nature Communications - 2018 -
Mitochondrial DNA replication in mammalian cells: overview of the
pathway
Maria Falkenberg
Essays in Biochemistry - 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 -
A multi-systemic mitochondrial disorder due to a dominant p.Y955H disease variant in DNA polymerase
gamma
Triinu Siibak, P. Clemente, A. Bratic, H. Bruhn, T. E. S. Kauppila, Bertil Macao, F. A. Schober, N. Lesko, R. Wibom, K. Naess, I. Nennesmo, A. Wedell, Bradley Peter, C. Freyer, Maria Falkenberg
Human Molecular Genetics - 2017 -
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 -
Selective mitochondrial DNA degradation following double-strand
breaks
A. Moretton, F. Morel, Bertil Macao, P. Lachaume, L. Ishak, M. Lefebvre, I. Garreau-Balandier, P. Vernet, Maria Falkenberg, G. Farge
Plos One - 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 -
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 -
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 -
Complementation between polymerase- and exonuclease-deficient mitochondrial DNA polymerase mutants in genomically engineered
flies
A. Bratic, T. E. S. Kauppila, Bertil Macao, S. Grönke, Triinu Siibak, J. B. Stewart, F. Baggio, J. Dols, L. Partridge, Maria Falkenberg, A. Wredenberg, N. G. Larsson
Nature Communications - 2015 -
Cross-strand binding of TFAM to a single mtDNA molecule forms the mitochondrial
nucleoid
C. Kukat, K. M. Davies, C. A. Wurm, H. Spahr, N. A. Bonekamp, I. Kuhl, F. Joos, P. L. Polosa, C. B. Park, Viktor Posse, Maria Falkenberg, S. Jakobs, W. Kuhlbrandt, N. G. Larsson
Proceedings of the National Academy of Sciences of the United States of America - 2015 -
Primer removal during mammalian mitochondrial DNA
replication
Jay (Jennifer) Uhler, Maria Falkenberg
DNA Repair - 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 -
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 -
A human mitochondrial poly(A) polymerase mutation reveals the complexities of post-transcriptional mitochondrial gene
expression
W. C. Wilson, H. T. Hornig-Do, F. Bruni, J. H. Chang, A. A. Jourdain, J. C. Martinou, Maria Falkenberg, H. Spahr, N. G. Larsson, R. J. Lewis, L. Hewitt, A. Basle, H. E. Cross, L. Tong, R. R. Lebel, A. H. Crosby, Z. M. A. Chrzanowska-Lightowlers, R. N. Lightowlers
Human Molecular Genetics - 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 -
Subnormal levels of POLgA cause inefficient initiation of light-strand DNA synthesis and lead to mitochondrial DNA deletions and progressive external
ophthalmoplegia
Sara Roos, Bertil Macao, Javier Miralles Fusté, Christopher Lindberg, Elisabeth Jemt, Elisabeth Holme, Ali-Reza Moslemi, Anders Oldfors, Maria Falkenberg
Human Molecular Genetics - 2013 -
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 Leucine-rich Pentatricopeptide Repeat-containing Protein (LRPPRC) Does Not Activate Transcription in Mammalian
Mitochondria
J. Harmel, B. Ruzzenente, M. Terzioglu, H. Spahr, Maria Falkenberg, N. G. Larsson
Journal of Biological Chemistry - 2013 -
TWINKLE is an essential mitochondrial helicase required for synthesis of nascent D-loop strands and complete mtDNA
replication
D. Milenkovic, S. Matic, I. Kuhl, B. Ruzzenente, C. Freyer, Elisabeth Jemt, C. B. Park, Maria Falkenberg, N. G. Larsson
Human Molecular Genetics - 2013 -
MtSSB may sequester UNG1 at mitochondrial ssDNA and delay uracil processing until the dsDNA conformation is
restored
Kristian Wollen Steen, Berit Doseth, Marianne P. Westbye, Mansour Akbari, Dongchon Kang, Maria Falkenberg, Geir Slupphaug
DNA Repair - 2012 -
Mammalian transcription factor A is a core component of the mitochondrial transcription
machinery
Yonghong Shi, Anke Dierckx, Paulina Wanrooij, Sjoerd Wanrooij, N. G. Larsson, Marcus Wilhelmsson, Maria Falkenberg, Claes M Gustafsson
Proceedings of the National Academy of Science of the United States of America - 2012 -
A hybrid G-quadruplex structure formed between RNA and DNA explains the extraordinary stability of the mitochondrial
R-loop
Paulina Wanrooij, Jay (Jennifer) Uhler, Yonghong Shi, Fredrik Westerlund, Maria Falkenberg, Claes M Gustafsson
Nucleic Acids Research - 2012 -
Role of human DNA glycosylase Nei-like 2 (NEIL2) and single strand break repair protein polynucleotide kinase 3'-phosphatase in maintenance of mitochondrial
genome.
Santi M Mandal, Muralidhar L Hegde, Arpita Chatterjee, Pavana M Hegde, Bartosz Szczesny, Dibyendu Banerjee, Istvan Boldogh, Rui Gao, Maria Falkenberg, Claes M Gustafsson, Partha S Sarkar, Tapas K Hazra
The Journal of biological chemistry - 2012 -
In vivo mutagenesis reveals that OriL is essential for mitochondrial DNA
replication.
Sjoerd Wanrooij, Javier Miralles Fusté, James B Stewart, Paulina H Wanrooij, Tore Samuelsson, Nils-Göran Larsson, Claes M Gustafsson, Maria Falkenberg
EMBO reports - 2012 -
Protein sliding and DNA denaturation are essential for DNA organization by human mitochondrial transcription factor
A
G. Farge, N. Laurens, O. D. Broekmans, Smjl van den Wildenberg, L. C. M. Dekker, M. Gaspari, Claes M Gustafsson, E. J. G. Peterman, Maria Falkenberg, G. J. L. Wuite
Nature Communications - 2012 -
The mitochondrial DNA helicase TWINKLE can assemble on a closed circular template and support initiation of DNA
synthesis
Elisabeth Jemt, G Farge, Stefan Bäckström, T Holmlund, Claes M Gustafsson, Maria Falkenberg
NUCLEIC ACIDS RESEARCH - 2011 -
Sequence-specific stalling of DNA polymerase gamma and the effects of mutations causing progressive
ophthalmoplegia
N Atanassova, Javier Miralles Fusté, Sjoerd Wanrooij, Bertil Macao, S Goffart, Stefan Bäckström, G Farge, I Khvorostov, NG Larsson, JN Spelbrink, Maria Falkenberg
Human Molecular Genetics - 2011 -
Adenosine Kinase Deficiency Disrupts the Methionine Cycle and Causes Hypermethioninemia, Encephalopathy, and Abnormal Liver
Function
Magnus K. Bjursell, HJ Blom, Jorge Asin-Cayuela, ML Engvall, N Lesko, S Balasubramaniam, G Brandberg, M Halldin, Maria Falkenberg, C Jakobs, D Smith, E Struys, U von Dobeln, Claes M Gustafsson, J Lundeberg, A Wedell
AMERICAN JOURNAL OF HUMAN GENETICS - 2011 -
Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of
mtDNA
C Kukat, CA Wurm, H Spahr, Maria Falkenberg, NG Larsson, S Jakobs
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA - 2011 -
Human mitochondrial transcription revisited: only TFAM and TFB2M are required for transcription of the mitochondrial genes in
vitro.
Dmitry Litonin, Marina Sologub, Yonghong Shi, Maria Savkina, Michael Anikin, Maria Falkenberg, Claes M Gustafsson, Dmitry Temiakov
The Journal of biological chemistry - 2010 -
Maintenance of respiratory chain function in mouse hearts with severely impaired mtDNA
transcription
Christoph Freyer, Chan Bae Park, Mats I. Ekstrand, Yonghong Shi, Julia Khvorostova, Rolf Wibom, Maria Falkenberg, Claes M Gustafsson, Nils-Goran Larsson
NUCLEIC ACIDS RESEARCH - 2010 -
G-quadruplex structures in RNA stimulate mitochondrial transcription termination and primer
formation
Paulina Wanrooij, Jay (Jennifer) Uhler, Tomas Simonsson, Maria Falkenberg, Claes M Gustafsson
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA - 2010 -
The human mitochondrial replication fork in health and
disease.
Sjoerd Wanrooij, Maria Falkenberg
Biochimica et biophysica acta - 2010 -
Mitochondrial RNA polymerase is needed for activation of the origin of light-strand DNA
replication.
Javier Miralles Fusté, Sjoerd Wanrooij, Elisabeth Jemt, Caroline E Granycome, Tricia J Cluett, Yonghong Shi, Neli Atanassova, Ian J Holt, Claes M Gustafsson, Maria Falkenberg
Molecular cell - 2010 -
Structure-function defects of the twinkle amino-terminal region in progressive external
ophthalmoplegia.
Teresa Holmlund, Géraldine Farge, Vineet Pande, Jenny Korhonen, L Nilsson, Maria Falkenberg
Biochimica et biophysica acta - 2009 -
Unraveling the structure of DNA during overstretching by using multicolor, single-molecule fluorescence
imaging.
Joost van Mameren, Peter Gross, Geraldine Farge, Pleuni Hooijman, Mauro Modesti, Maria Falkenberg, Gijs J L Wuite, Erwin J G Peterman
Proceedings of the National Academy of Sciences of the United States of America - 2009 -
Structure casts light on mtDNA
replication.
Maria Falkenberg, Nils-Göran Larsson
Cell - 2009 -
The N-terminal domain of TWINKLE contributes to single-stranded DNA binding and DNA helicase
activities.
Géraldine Farge, Teresa Holmlund, Julia Khvorostova, Reza Rofougaran, Anders Hofer, Maria Falkenberg
Nucleic acids research - 2008 -
Structure-function defects of the TWINKLE linker region in progressive external
ophthalmoplegia.
Jenny A Korhonen, Vineet Pande, Teresa Holmlund, Géraldine Farge, Xuan Hoi Pham, Lennart Nilsson, Maria Falkenberg
Journal of molecular biology - 2008 -
Human mitochondrial RNA polymerase primes lagging-strand DNA synthesis in
vitro
Sjoerd Wanrooij, Javier Miralles Fuste, Geraldine Farge, Yonghong Shi, Claes M Gustafsson, Maria Falkenberg
Proceedings of The National Academy of Sciences of The United States of America - 2008 -
MTERF3 is a negative regulator of mammalian mtDNA
transcription.
Chan Bae Park, Jorge Asin-Cayuela, Yolanda Cámara, Yonghong Shi, Mina Pellegrini, Martina Gaspari, Rolf Wibom, Kjell Hultenby, Hediye Erdjument-Bromage, Paul Tempst, Maria Falkenberg, Claes M Gustafsson, Nils-Göran Larsson
Cell - 2007 -
The accessory subunit B of DNA polymerase gamma is required for mitochondrial replisome
function.
Géraldine Farge, Xuan Hoi Pham, Teresa Holmlund, Ivan Khorostov, Maria Falkenberg
Nucleic acids research - 2007 -
Cloning of the sea urchin mitochondrial RNA polymerase and reconstitution of the transcription termination
system.
Paola Loguercio Polosa, Stefania Deceglie, Maria Falkenberg, Marina Roberti, Barbara Di Ponzio, Maria Nicola Gadaleta, Palmiro Cantatore
Nucleic acids research - 2007 -
DNA replication and transcription in mammalian
mitochondria.
Maria Falkenberg, Nils-Göran Larsson, Claes M Gustafsson
Annual review of biochemistry - 2007 -
Conserved sequence box II directs transcription termination and primer formation in
mitochondria.
Xuan Hoi Pham, Géraldine Farge, Yonghong Shi, Martina Gaspari, Claes M Gustafsson, Maria Falkenberg
The Journal of biological chemistry - 2006 -
A family of putative transcription termination factors shared amongst metazoans and
plants.
Tomas Linder, Chan Bae Park, Jordi Asin-Cayuela, Mina Pellegrini, Nils-Göran Larsson, Maria Falkenberg, Tore Samuelsson, Claes M Gustafsson
Current genetics - 2005 -
Premature ageing in mice expressing defective mitochondrial DNA
polymerase
A. Trifunovic, A. Wredenberg, Maria Falkenberg, J. N. Spelbrink, A. T. Rovio, C. E. Bruder, Mohammad Bohlooly-Yeganeh, S. Gidlof, Anders Oldfors, R. Wibom, J. Tornell, H. T. Jacobs, N. G. Larsson
Nature - 2004 -
Mitochondrial transcription factor A regulates mtDNA copy number in
mammals.
Mats I Ekstrand, Maria Falkenberg, Anja Rantanen, Chan Bae Park, Martina Gaspari, Kjell Hultenby, Pierre Rustin, Claes M Gustafsson, Nils-Göran Larsson
Human molecular genetics - 2004 -
Reconstitution of a minimal mtDNA replisome in
vitro.
Jenny A Korhonen, Xuan Hoi Pham, Mina Pellegrini, Maria Falkenberg
The EMBO journal - 2004 -
The mitochondrial RNA polymerase contributes critically to promoter specificity in mammalian
cells.
Martina Gaspari, Maria Falkenberg, Nils-Göran Larsson, Claes M Gustafsson
The EMBO journal - 2004 -
Characterization of the mouse genes for mitochondrial transcription factors B1 and
B2.
Anja Rantanen, Martina Gaspari, Maria Falkenberg, Claes M Gustafsson, Nils-Göran Larsson
Mammalian genome : official journal of the International Mammalian Genome Society - 2003 -
TWINKLE Has 5' -> 3' DNA helicase activity and is specifically stimulated by mitochondrial single-stranded DNA-binding
protein.
Jenny A Korhonen, Martina Gaspari, Maria Falkenberg
The Journal of biological chemistry - 2003 -
Mitochondrial transcription factors B1 and B2 activate transcription of human
mtDNA.
Maria Falkenberg, Martina Gaspari, Anja Rantanen, Aleksandra Trifunovic, Nils-Göran Larsson, Claes M Gustafsson
Nature genetics - 2002 -
Rep-dependent initiation of adeno-associated virus type 2 DNA replication by a herpes simplex virus type 1 replication complex in a reconstituted
system.
P Ward, Maria Falkenberg, Per Elias, M Weitzman, R M Linden
Journal of virology - 2001 -
Leading and lagging strand DNA synthesis in vitro by a reconstituted herpes simplex virus type 1
replisome.
Maria Falkenberg, I R Lehman, Per Elias
Proceedings of the National Academy of Sciences of the United States of America - 2000 -
The herpes simplex virus type 1 helicase-primase. Analysis of helicase
activity.
Maria Falkenberg, Per Elias, I R Lehman
The Journal of biological chemistry - 1998 -
The UL8 subunit of the heterotrimeric herpes simplex virus type 1 helicase-primase is required for the unwinding of single strand DNA-binding protein (ICP8)-coated DNA
substrates.
Maria Falkenberg, D A Bushnell, Per Elias, I R Lehman
The Journal of biological chemistry - 1997 -
The DNA ligands influence the interactions between the herpes simplex virus 1 origin binding protein and the single strand DNA-binding protein,
ICP-8.
Claes M Gustafsson, Maria Falkenberg, Stina Simonsson, Hadi Valadi, Per Elias
The Journal of biological chemistry - 1995