Steffi Goffart

Analysis of Mitochondrial DNA Replication by Two-Dimensional Agarose Gel Electrophoresis

Goffart, Steffi; Pohjoismäki, Jaakko. Teoksessa: Nicholls, TJ; Uhler, JP; Falkenberg, M(toim.) , 2023. Mitochondrial DNA. s. 241-266. Humana press

Uncharacterized protein C17orf80 - a novel interactor of human mitochondrial nucleoids

Potter, Alisa; Hangas, Anu; Goffart, Steffi; Huynen, Martijn A; Cabrera-Orefice, Alfredo; Spelbrink, Johannes N. 2023. Journal of cell science. 136: . jcs260822

Adaptive and Pathological Outcomes of Radiation Stress-Induced Redox Signaling

Pohjoismäki, Jaakko LO; Goffart, Steffi. 2022. Antioxidants and redox signaling. 37: 336-348

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

Paul, Dhiraj; Paasisalo, Inga; Katainen, Markku; Goffart, Steffi; Pumpanen, Jukka; Siljanen, Henri M. P.. 2022. Microbiology resource announcements. 11: . e0090722

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

Rahman, Md. Mostafijur; Young, Carolyn K.J; Goffart, Steffi; Pohjoismäki, Jaakko L.O; Young, Matthew J. 2022. Journal of biological chemistry. 298:

Molecular phenotyping uncovers differences in basic housekeeping functions among closely related species of hares (Lepus spp., Lagomorpha: Leporidae)

Gaertner, Kateryna; Michell, Craig; Tapanainen, Riikka; Goffart, Steffi; Saari, Sina; Soininmäki, Manu; Dufour, Eric; Pohjoismäki, Jaakko LO. 2022. Molecular ecology. 2023; 32: 4097-4117

Top3α is the replicative topoisomerase in mitochondrial DNA replication

Hangas, Anu; Kekäläinen, Nina J; Potter, Alisa; Michell, Craig; Aho, Kauko J; Rutanen, Chiara; Spelbrink, Johannes N; Pohjoismäki, Jaakko L; Goffart, Steffi. 2022. Nucleic acids research. 50: 8733-8748

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

Luzwick, Jessica W.; Dombi, Eszter; Boisvert, Rebecca A.; Roy, Sunetra; Park, Soyoung; Kunnimalaiyaan, Selvi; Goffart, Steffi; Schindler, Detlev; Schlacher, Katharina. 2021. Science advances. 7: eabf9441

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

Aasumets, Koit; Basikhina, Yuliya; Pohjoismäki, Jaakko L; Goffart, Steffi; Gerhold, Joachim. 2021. Biochemistry and biophysics reports. 28: 101142

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

Goffart, S; Tikkanen, P; Michell, C; Wilson, T; Pohjoismäki, JLO. 2021. Cells. 10: 1075

Reply to: Proofreading deficiency in mitochondrial DNA polymerase does not affect total dNTP pools in mouse embryos

Hämäläinen, Riikka H; Landoni, Juan C; Ahlqvist, Kati J; Goffart, Steffi; Ryytty, Sanna; Rahman, M Obaidur; Brilhante, Virginia; Icay, Katherine; Hautaniemi, Sampsa; Wang, Liya; Laiho, Marikki; Suomalainen, Anu. 2020. Nature metabolism. 2: 676-677

Defects in mtDNA replication challenge nuclear genome stability through nucleotide depletion and provide a unifying mechanism for mouse progerias

Hämäläinen, Riikka H; Landoni, Juan C; Ahlqvist, Kati J; Goffart, Steffi; Ryytty, Sanna; Rahman, M Obaidur; Brilhante, Virginia; lcay, Katherine; Hautaniemi, Sampsa; Wang, Liya; Laiho, Marikki; Suomalainen, Anu. 2019. Nature metabolism. 1: 958-965

Hydraulically pressed silver nanowire-cotton fibers as an active platform for filtering and surface-enhanced Raman scattering detection of bacteria from fluid

Ankudze, Bright; Asare, Bismark; Goffart, Steffi; Koistinen, Arto; Nuutinen, Tarmo; Matikainen, Antti; Andoh, Sampson Saj; Roussey, Matthieu;, Pakkanen, Tuula. T. 2019. Applied surface science. 479: 663-668

Replication fork rescue in mammalian mitochondria

Torregrosa-Muñumer, R; Hangas, A; Goffart, S; Blei, D; Zsurka, G; Griffith, J; Kunz ,WS; Pohjoismäki, JLO. 2019. Scientific reports. 9: 8785

Twist and Turn - Topoisomerase Functions in Mitochondrial DNA Maintenance

Goffart, S; Hangas, A; Pohjoismäki, JLO. 2019. International journal of molecular sciences. 20: 2041

ATAD3 controls mitochondrial cristae structure, influencing mtDNA replication and cholesterol levels in muscle

Peralta, Susana; Goffart, Steffi; Williams, Sion L; Diaz, Francisca; Garcia, Sofia; Nissanka, Nadee; Area-Gomez, Estela; Pohjoismäki, Jaakko; Moraes, Carlos T. 2018. Journal of cell science. 131: jcs217075

Ciprofloxacin impairs mitochondrial DNA replication initiation through inhibition of Topoisomerase 2

Hangas, Anu; Aasumets, Koit; Kekäläinen, Nina J; Paloheinä, Mika; Pohjoismäki, Jaakko L; Gerhold, Joachim M; Goffart, Steffi. 2018. Nucleic acids research. 46: 9625-9636

Known Unknowns of Mammalian Mitochondrial DNA Maintenance

Pohjoismäki, Jaakko LO; Forslund, Josefin ME; Goffart, Steffi; Torregrosa-Muñumer, Ruben; Wanrooij, Sjoerd. 2018. Bioessays. 40: 1800102

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

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 FM. 2018. Molecular therapy. Methods & clinical development. 8: 152-165

Chemoproteomic, biochemical and pharmacological approaches in the discovery of inhibitors targeting human alfa/beta-hydrolase domain containing 11 (ABHD11)

Navia-Paldanius D, Patel JZ, López Navarro M, Jakupovic H, Goffart S, Pasonen-Seppänen S, Nevalainen TJ, Jääskeläinen T, Laitinen T, Laitinen JT, Savinainen JR. 2016. European journal of pharmaceutical sciences. 93: 253-263

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

Torregrosa-Muñumer Ruben, Goffart Steffi, Haikonen Juha A, Pohjoismäki Jaakko LO. 2015. Molecular biology of the cell. 26: 4197-4208

Binding to G-quadruplex RNA activates the mitochondrial GTPase NOA1

Al-Furoukh Natalie, Goffart Steffi, Szibor Marten, Wanrooij Sjoerd, Braun Thomas. 2013. Biochimica et biophysica acta: molecular cell research. 1833: 2933-2942

Overexpression of Twinkle-helicase protects cardiomyocytes from genotoxic stress caused by reactive oxygen species

Pohjoismäki JL, Williams SL, Boettger T, Goffart S, Kim J, Suomalainen A, Moraes CT, Braun T. 2013. Proceedings of the National Academy of Sciences of the United States of America. [Epub ahead of print]: x-x

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

Pohjoismäki JL, Boettger T, Liu Z, Goffart S, Szibor M, Braun T. 2012. Nucleic acids research. 40: 6595-607

Of circles, forks and humanity: Topological organisation and replication of mammalian mitochondrial DNA.

Pohjoismäki JL, Goffart S. 2011. Bioessays. 33: 290-9

Replication stalling by catalytically impaired Twinkle induces mitochondrial DNA rearrangements in cultured cells.

Pohjoismäki JL, Goffart S, Spelbrink JN.. 2011. Mitochondrion. 11: 630-4

Sequence-specific stalling of DNA polymerase &#947 and the effects of mutations causing progressive ophthalmoplegia

Atanassova N, Fusté JM, Wanrooij S, Macao B, Goffart S, Bäckström S, Farge G, Khvorostov I, Larsson NG, Spelbrink JN, Falkenberg M. 2011. Human molecular genetics. 20: 1212-23

Developmental and pathological changes in the human cardiac muscle mitochondrial DNA organization, replication and copy number

Pohjoismäki JL, Goffart S, Taylor RW, Turnbull DM, Suomalainen A, Jacobs HT, Karhunen PJ.. 2010. PLoS ONE. 5: e10426

Mammalian mitochondrial DNA replication intermediates are essentially duplex but contain extensive tracts of RNA/DNA hybrid.

Pohjoismäki JL, Holmes JB, Wood SR, Yang MY, Yasukawa T, Reyes A, Bailey LJ, Cluett TJ, Goffart S, Willcox S, Rigby RE, Jackson AP, Spelbrink JN, Griffith JD, Crouch RJ, Jacobs HT, Holt IJ.. 2010. Journal of molecular biology. 397: 1144-55

Adaptation of topoisomerase I paralogs to nuclear and mitochondrial DNA.

Dalla Rosa I, Goffart S, Wurm M, Wiek C, Essmann F, Sobek S, Schroeder P, Zhang H, Krutmann J, Hanenberg H, Schulze-Osthoff K, Mielke C, Pommier Y, Boege F, Christensen MO.. 2009. Nucleic acids research. 37: 6414-28

Human heart mitochondrial DNA is organized in complex catenated networks containing abundant four-way junctions and replication forks.

Pohjoismäki JL, Goffart S, Tyynismaa H, Willcox S, Ide T, Kang D, Suomalainen A, Karhunen PJ, Griffith JD, Holt IJ, Jacobs HT. 2009. Journal of biological chemistry. 284: 21446-57

Inducible expression in human cells, purification, and in vitro assays for the mitochondrial DNA helicase Twinkle.

Goffart S, Spelbrink H. 2009. Methods in molecular biology. 554: 103-19

The accessory subunit of mitochondrial DNA polymerase gamma determines the DNA content of mitochondrial nucleoids in human cultured cells.

Di Re M, Sembongi H, He J, Reyes A, Yasukawa T, Martinsson P, Bailey LJ, Goffart S, Boyd-Kirkup JD, Wong TS, Fersht AR, Spelbrink JN, Holt IJ.. 2009. Nucleic acids research. 37: 5701-13

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

Goffart S, Cooper HM, Tyynismaa H, Wanrooij S, Suomalainen A, Spelbrink JN.. 2009. Human molecular genetics. 18: 328-40

CREB-1alpha is recruited to and mediates upregulation of the cytochrome c promoter during enhanced mitochondrial biogenesis accompanying skeletal muscle differentiation.

Franko A, Mayer S, Thiel G, Mercy L, Arnould T, Hornig-Do HT, Wiesner RJ, Goffart S. 2008. Molecular and cellular biology. 28: 2446-59

Infantile-onset spinocerebellar ataxia and mitochondrial recessive ataxia syndrome are associated with neuronal complex I defect and mtDNA depletion.

Hakonen AH, Goffart S, Marjavaara S, Paetau A, Cooper H, Mattila K, Lampinen M, Sajantila A, Lönnqvist T, Spelbrink JN, Suomalainen A. 2008. Human molecular genetics. 17: 3822-35

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

Wanrooij S, Goffart S, Pohjoismäki JL, Yasukawa T, Spelbrink JN.. 2007. Nucleic acids research. 35: 3238-51

The mitochondria of cultured mammalian cells: I. Analysis by immunofluorescence microscopy, histochemistry, subcellular fractionation, and cell fusion.

Malka F, Auré K, Goffart S, Spelbrink JN, Rojo M.. 2007. Methods in molecular biology. 372: 3-16

The mitochondria of cultured mammalian cells: II. Expression and visualization of exogenous proteins in fixed and live cells.

Goffart S, Martinsson P, Malka F, Rojo M, Spelbrink JN.. 2007. Methods in molecular biology. 372: 17-32

Alpha-actinin 4 and BAT1 interaction with the cytochrome c promoter upon skeletal muscle differentiation.

Goffart S, Franko A, Clemen CS, Wiesner RJ.. 2006. Current genetics. 49: 125-35

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

Pohjoismäki JL, Wanrooij S, Hyvärinen AK, Goffart S, Holt IJ, Spelbrink JN, Jacobs HT. 2006. Nucleic acids research. 34: 5815-28

Regulation of mitochondrial proliferation in the heart: power-plant failure contributes to cardiac failure in hypertrophy.

Goffart S, von Kleist-Retzow JC, Wiesner RJ.. 2004. Cardiovascular research. 64: 198-207

Transient overexpression of mitochondrial transcription factor A (TFAM) is sufficient to stimulate mitochondrial DNA transcription, but not sufficient to increase mtDNA copy number in cultured cells.

Maniura-Weber K, Goffart S, Garstka HL, Montoya J, Wiesner RJ.. 2004. Nucleic acids research. 32: 6015-27

Regulation and co-ordination of nuclear gene expression during mitochondrial biogenesis.

Goffart S, Wiesner RJ.. 2003. Experimental physiology. 88: 33-40

Relocation of urf a from the mitochondrion to the nucleus cures the mitochondrial mutator phenotype in the fission yeast Schizosaccharomyces pombe.

Neu R, Goffart S, Wolf K, Schäfer B. 1998. MOLECULAR AND GENERAL GENETICS (BERLIN). 258: 389-96

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Research groups

Analysis of Mitochondrial DNA Replication by Two-Dimensional Agarose Gel Electrophoresis

Uncharacterized protein C17orf80 - a novel interactor of human mitochondrial nucleoids

Adaptive and Pathological Outcomes of Radiation Stress-Induced Redox Signaling

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

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

Molecular phenotyping uncovers differences in basic housekeeping functions among closely related species of hares (Lepus spp., Lagomorpha: Leporidae)

Top3α is the replicative topoisomerase in mitochondrial DNA replication

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

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

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

Reply to: Proofreading deficiency in mitochondrial DNA polymerase does not affect total dNTP pools in mouse embryos

Defects in mtDNA replication challenge nuclear genome stability through nucleotide depletion and provide a unifying mechanism for mouse progerias

Hydraulically pressed silver nanowire-cotton fibers as an active platform for filtering and surface-enhanced Raman scattering detection of bacteria from fluid

Replication fork rescue in mammalian mitochondria

Twist and Turn - Topoisomerase Functions in Mitochondrial DNA Maintenance

ATAD3 controls mitochondrial cristae structure, influencing mtDNA replication and cholesterol levels in muscle

Ciprofloxacin impairs mitochondrial DNA replication initiation through inhibition of Topoisomerase 2

Known Unknowns of Mammalian Mitochondrial DNA Maintenance

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

Tissue specific differences in mitochondrial DNA maintenance and expression

PrimPol is required for replication reinitiation after mtDNA damage

The role of mitochondria in cardiac development and protection

Chemoproteomic, biochemical and pharmacological approaches in the discovery of inhibitors targeting human alfa/beta-hydrolase domain containing 11 (ABHD11)

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

Binding to G-quadruplex RNA activates the mitochondrial GTPase NOA1

Overexpression of Twinkle-helicase protects cardiomyocytes from genotoxic stress caused by reactive oxygen species

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

Of circles, forks and humanity: Topological organisation and replication of mammalian mitochondrial DNA.

Replication stalling by catalytically impaired Twinkle induces mitochondrial DNA rearrangements in cultured cells.

Sequence-specific stalling of DNA polymerase &#947 and the effects of mutations causing progressive ophthalmoplegia

Developmental and pathological changes in the human cardiac muscle mitochondrial DNA organization, replication and copy number

Mammalian mitochondrial DNA replication intermediates are essentially duplex but contain extensive tracts of RNA/DNA hybrid.

Adaptation of topoisomerase I paralogs to nuclear and mitochondrial DNA.

Human heart mitochondrial DNA is organized in complex catenated networks containing abundant four-way junctions and replication forks.

Inducible expression in human cells, purification, and in vitro assays for the mitochondrial DNA helicase Twinkle.

The accessory subunit of mitochondrial DNA polymerase gamma determines the DNA content of mitochondrial nucleoids in human cultured cells.

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

CREB-1alpha is recruited to and mediates upregulation of the cytochrome c promoter during enhanced mitochondrial biogenesis accompanying skeletal muscle differentiation.

Infantile-onset spinocerebellar ataxia and mitochondrial recessive ataxia syndrome are associated with neuronal complex I defect and mtDNA depletion.

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

The mitochondria of cultured mammalian cells: I. Analysis by immunofluorescence microscopy, histochemistry, subcellular fractionation, and cell fusion.

The mitochondria of cultured mammalian cells: II. Expression and visualization of exogenous proteins in fixed and live cells.

Alpha-actinin 4 and BAT1 interaction with the cytochrome c promoter upon skeletal muscle differentiation.

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

Regulation of mitochondrial proliferation in the heart: power-plant failure contributes to cardiac failure in hypertrophy.

Transient overexpression of mitochondrial transcription factor A (TFAM) is sufficient to stimulate mitochondrial DNA transcription, but not sufficient to increase mtDNA copy number in cultured cells.

Regulation and co-ordination of nuclear gene expression during mitochondrial biogenesis.

Relocation of urf a from the mitochondrion to the nucleus cures the mitochondrial mutator phenotype in the fission yeast Schizosaccharomyces pombe.