Twist and Turn—Topoisomerase Functions in Mitochondrial DNA Maintenance

por Goffart, Steffi, Hangas, Anu, Pohjoismäki, Jaakko L. O.
Publicado 2019

TFAM knockdown-triggered mtDNA-nucleoid aggregation and a decrease in mtDNA copy number induce the reorganization of nucleoid populations and mitochondria-associated ER-membrane co...

por Aasumets, Koit, Basikhina, Yuliya, Pohjoismäki, Jaakko L., Goffart, Steffi, Gerhold, Joachim
Publicado 2021

Low doses of ultraviolet radiation and oxidative damage induce dramatic accumulation of mitochondrial DNA replication intermediates, fork regression, and replication initiation shi...

por Torregrosa-Muñumer, Rubén, Goffart, Steffi, Haikonen, Juha A., Pohjoismäki, Jaakko L. O.
Publicado 2015

The Type and Source of Reactive Oxygen Species Influences the Outcome of Oxidative Stress in Cultured Cells

por Goffart, Steffi, Tikkanen, Petra, Michell, Craig, Wilson, Trevor, Pohjoismäki, Jaakko L. O.
Publicado 2021

Expression of catalytic mutants of the mtDNA helicase Twinkle and polymerase POLG causes distinct replication stalling phenotypes

por Wanrooij, Sjoerd, Goffart, Steffi, Pohjoismäki, Jaakko L.O., Yasukawa, Takehiro, Spelbrink, Johannes N.
Publicado 2007

Twinkle mutations associated with autosomal dominant progressive external ophthalmoplegia lead to impaired helicase function and in vivo mtDNA replication stalling

por Goffart, Steffi, Cooper, Helen M., Tyynismaa, Henna, Wanrooij, Sjoerd, Suomalainen, Anu, Spelbrink, Johannes N.
Publicado 2009

Oxidative stress during mitochondrial biogenesis compromises mtDNA integrity in growing hearts and induces a global DNA repair response

por Pohjoismäki, Jaakko L. O., Boettger, Thomas, Liu, Zhipei, Goffart, Steffi, Szibor, Marten, Braun, Thomas
Publicado 2012

Draft Genome Sequence of Bradyrhizobium denitrificans K2, Cultivated from an Air Ventilation Environment

por Paul, Dhiraj, Paasisalo, Inga, Katainen, Markku, Goffart, Steffi, Pumpanen, Jukka, Siljanen, Henri M. P.
Publicado 2022

Uncharacterized protein C17orf80 – a novel interactor of human mitochondrial nucleoids

por Potter, Alisa, Hangas, Anu, Goffart, Steffi, Huynen, Martijn A., Cabrera-Orefice, Alfredo, Spelbrink, Johannes N.
Publicado 2023

Heterozygous p.Y955C mutation in DNA polymerase γ leads to alterations in bioenergetics, complex I subunit expression, and mtDNA replication

por Rahman, Md. Mostafijur, Young, Carolyn K.J., Goffart, Steffi, Pohjoismäki, Jaakko L.O., Young, Matthew J.
Publicado 2022

Ciprofloxacin impairs mitochondrial DNA replication initiation through inhibition of Topoisomerase 2

por Hangas, Anu, Aasumets, Koit, Kekäläinen, Nina J, Paloheinä, Mika, Pohjoismäki, Jaakko L, Gerhold, Joachim M, Goffart, Steffi
Publicado 2018

Alterations to the expression level of mitochondrial transcription factor A, TFAM, modify the mode of mitochondrial DNA replication in cultured human cells

por Pohjoismäki, Jaakko L. O., Wanrooij, Sjoerd, Hyvärinen, Anne K., Goffart, Steffi, Holt, Ian J., Spelbrink, Johannes N., Jacobs, Howard T.
Publicado 2006

Developmental and Pathological Changes in the Human Cardiac Muscle Mitochondrial DNA Organization, Replication and Copy Number

por Pohjoismäki, Jaakko L. O., Goffart, Steffi, Taylor, Robert W., Turnbull, Douglas M., Suomalainen, Anu, Jacobs, Howard T., Karhunen, Pekka J.
Publicado 2010

Replication fork rescue in mammalian mitochondria

por Torregrosa-Muñumer, Rubén, Hangas, Anu, Goffart, Steffi, Blei, Daniel, Zsurka, Gábor, Griffith, Jack, Kunz, Wolfram S., Pohjoismäki, Jaakko L. O.
Publicado 2019

MRE11-dependent instability in mitochondrial DNA fork protection activates a cGAS immune signaling pathway

por Luzwick, Jessica W., Dombi, Eszter, Boisvert, Rebecca A., Roy, Sunetra, Park, Soyoung, Kunnimalaiyaan, Selvi, Goffart, Steffi, Schindler, Detlev, Schlacher, Katharina
Publicado 2021

Top3α is the replicative topoisomerase in mitochondrial DNA replication

por Hangas, Anu, Kekäläinen, Nina J, Potter, Alisa, Michell, Craig, Aho, Kauko J, Rutanen, Chiara, Spelbrink, Johannes N, Pohjoismäki, Jaakko L, Goffart, Steffi
Publicado 2022

Human Heart Mitochondrial DNA Is Organized in Complex Catenated Networks Containing Abundant Four-way Junctions and Replication Forks

por Pohjoismäki, Jaakko L. O., Goffart, Steffi, Tyynismaa, Henna, Willcox, Smaranda, Ide, Tomomi, Kang, Dongchon, Suomalainen, Anu, Karhunen, Pekka J., Griffith, Jack D., Holt, Ian J., Jacobs, Howard T.
Publicado 2009

Adaptation of topoisomerase I paralogs to nuclear and mitochondrial DNA

por Rosa, Ilaria Dalla, Goffart, Steffi, Wurm, Melanie, Wiek, Constanze, Essmann, Frank, Sobek, Stefan, Schroeder, Peter, Zhang, Hongliang, Krutmann, Jean, Hanenberg, Helmut, Schulze-Osthoff, Klaus, Mielke, Christian, Pommier, Yves, Boege, Fritz, Christensen, Morten O.
Publicado 2009

Enhanced mitochondrial G-quadruplex formation impedes replication fork progression leading to mtDNA loss in human cells

por Doimo, Mara, Chaudhari, Namrata, Abrahamsson, Sanna, L’Hôte, Valentin, Nguyen, Tran V H, Berner, Andreas, Ndi, Mama, Abrahamsson, Alva, Das, Rabindra Nath, Aasumets, Koit, Goffart, Steffi, Pohjoismäki, Jaakko L O, López, Marcela Dávila, Chorell, Erik, Wanrooij, Sjoerd
Publicado 2023

Preclinical Efficacy and Safety Evaluation of Hematopoietic Stem Cell Gene Therapy in a Mouse Model of MNGIE

por Yadak, Rana, Cabrera-Pérez, Raquel, Torres-Torronteras, Javier, Bugiani, Marianna, Haeck, Joost C., Huston, Marshall W., Bogaerts, Elly, Goffart, Steffi, Jacobs, Edwin H., Stok, Merel, Leonardelli, Lorena, Biasco, Luca, Verdijk, Robert M., Bernsen, Monique R., Ruijter, George, Martí, Ramon, Wagemaker, Gerard, van Til, Niek P., de Coo, Irenaeus F.M.
Publicado 2018