STEFFI GOFFART

Contact info

RESEARCH GROUPS - UEF

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  Mitochondrial biology

Publications

• Goffart, S; Tikkanen, P; Michell, C; Wilson, T; Pohjoismäki, JLO. 2021. The Type and Source of Reactive Oxygen Species Influences the Outcome of Oxidative Stress in Cultured Cells Cells 10 5: 1075. 2021
• 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. Reply to: Proofreading deficiency in mitochondrial DNA polymerase does not affect total dNTP pools in mouse embryos Nature metabolism 2 8: 676-677. 2020
• Ankudze, Bright; Asare, Bismark; Goffart, Steffi; Koistinen, Arto; Nuutinen, Tarmo; Matikainen, Antti; Andoh, Sampson Saj; Roussey, Matthieu;, Pakkanen, Tuula. T. 2019. Hydraulically pressed silver nanowire-cotton fibers as an active platform for filtering and surface-enhanced Raman scattering detection of bacteria from fluid Applied surface science 479: 663-668. 2019
• Goffart, S; Hangas, A; Pohjoismäki, JLO. 2019. Twist and Turn - Topoisomerase Functions in Mitochondrial DNA Maintenance International journal of molecular sciences 20 8: 2041. 2019
• 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. Defects in mtDNA replication challenge nuclear genome stability through nucleotide depletion and provide a unifying mechanism for mouse progerias Nature metabolism 1: 958-965. 2019
• Torregrosa-Muñumer, R; Hangas, A; Goffart, S; Blei, D; Zsurka, G; Griffith, J; Kunz ,WS; Pohjoismäki, JLO. 2019. Replication fork rescue in mammalian mitochondria Scientific reports 9 1: 8785. 2019
• 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. Preclinical Efficacy and Safety Evaluation of Hematopoietic Stem Cell Gene Therapy in a Mouse Model of MNGIE. Molecular Therapy - Methods and Clinical Development 8: 152-165. 2018
• Pohjoismäki, Jaakko LO; Forslund, Josefin ME; Goffart, Steffi; Torregrosa-Muñumer, Ruben; Wanrooij, Sjoerd. 2018. Known Unknowns of Mammalian Mitochondrial DNA Maintenance BIOESSAYS 40 9: 1800102. 2018
• Peralta, Susana; Goffart, Steffi; Williams, Sion L; Diaz, Francisca; Garcia, Sofia; Nissanka, Nadee; Area-Gomez, Estela; Pohjoismäki, Jaakko; Moraes, Carlos T. 2018. ATAD3 controls mitochondrial cristae structure, influencing mtDNA replication and cholesterol levels in muscle JOURNAL OF CELL SCIENCE 131: jcs217075. 2018
• Herbers, Elena; Kekäläinen, Nina J; Hangas, Anu; Pohjoismäki, Jaakko L; Goffart, Steffi. 2018. Tissue specific differences in mitochondrial DNA maintenance and expression Mitochondrion 2019; 44: 85-92. 2018
• Hangas, Anu; Aasumets, Koit; Kekäläinen, Nina J; Paloheinä, Mika; Pohjoismäki, Jaakko L; Gerhold, Joachim M; Goffart, Steffi. 2018. Ciprofloxacin impairs mitochondrial DNA replication initiation through inhibition of Topoisomerase 2 NUCLEIC ACIDS RESEARCH 46 18: 9625-9636. 2018
• Pohjoismäki Jaakko L, Goffart Steffi. 2017. The role of mitochondria in cardiac development and protection. FREE RADICAL BIOLOGY & MEDICINE 106: 345-354. 2017
• Torregrosa-Muñumera Rubén, Forslund Josefin ME, Goffarta Steffi, Pfeiffer Annika, Stojkovic Gorazd, Carvalho Gustavo, Al-Furoukh Natalie, Blanco Luis, Wanrooij Sjoerd, Pohjoismäki Jaakko LO. 2017. PrimPol is required for replication reinitiation after mtDNA damage. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA 114 43: 11398-11403. 2017
• 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. Chemoproteomic, biochemical and pharmacological approaches in the discovery of inhibitors targeting human alfa/beta-hydrolase domain containing 11 (ABHD11). EUROPEAN JOURNAL OF PHARMACEUTICAL SCIENCES 93: 253-263. 2016
• Torregrosa-Muñumer Ruben, Goffart Steffi, Haikonen Juha A, Pohjoismäki Jaakko LO. 2015. Low doses of ultraviolet radiation and oxidative damage induce dramatic accumulation of mitochondrial DNA replication intermediates, fork regression, and replication initiation shift. MOLECULAR BIOLOGY OF THE CELL (BETHESDA MD) 26 23: 4197-4208. 2015
• Al-Furoukh Natalie, Goffart Steffi, Szibor Marten, Wanrooij Sjoerd, Braun Thomas. 2013. Binding to G-quadruplex RNA activates the mitochondrial GTPase NOA1. BIOCHIMICA ET BIOPHYSICA ACTA - MOLECULAR CELL RESEARCH 1833 12: 2933-2942. 2013
• Pohjoismäki JL, Williams SL, Boettger T, Goffart S, Kim J, Suomalainen A, Moraes CT, Braun T. 2013. Overexpression of Twinkle-helicase protects cardiomyocytes from genotoxic stress caused by reactive oxygen species. PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA [Epub ahead of print]: x-x. 2013
• Pohjoismäki JL, Boettger T, Liu Z, Goffart S, Szibor M, Braun T. 2012. Oxidative stress during mitochondrial biogenesis compromises mtDNA integrity in growing hearts and induces a global DNA repair response. NUCLEIC ACIDS RESEARCH 40 14: 6595-607. 2012
• Pohjoismäki JL, Goffart S. 2011. Of circles, forks and humanity: Topological organisation and replication of mammalian mitochondrial DNA.. BIOESSAYS 33 4: 290-9. 2011
• Pohjoismäki JL, Goffart S, Spelbrink JN.. 2011. Replication stalling by catalytically impaired Twinkle induces mitochondrial DNA rearrangements in cultured cells.. Mitochondrion 11 4: 630-4. 2011
• Atanassova N, Fusté JM, Wanrooij S, Macao B, Goffart S, Bäckström S, Farge G, Khvorostov I, Larsson NG, Spelbrink JN, Falkenberg M. 2011. Sequence-specific stalling of DNA polymerase γ and the effects of mutations causing progressive ophthalmoplegia. HUMAN MOLECULAR GENETICS 20 6: 1212-23. 2011
• Pohjoismäki JL, Goffart S, Taylor RW, Turnbull DM, Suomalainen A, Jacobs HT, Karhunen PJ.. 2010. Developmental and pathological changes in the human cardiac muscle mitochondrial DNA organization, replication and copy number. PLOS ONE [ONLINE] 5 5: e10426. 2010
• 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. Mammalian mitochondrial DNA replication intermediates are essentially duplex but contain extensive tracts of RNA/DNA hybrid. JOURNAL OF MOLECULAR BIOLOGY 397 5: 1144-55. 2010
• 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. Adaptation of topoisomerase I paralogs to nuclear and mitochondrial DNA.. NUCLEIC ACIDS RESEARCH 37 19: 6414-28. 2009
• 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. The accessory subunit of mitochondrial DNA polymerase gamma determines the DNA content of mitochondrial nucleoids in human cultured cells. NUCLEIC ACIDS RESEARCH 37 17: 5701-13. 2009
• Goffart S, Cooper HM, Tyynismaa H, Wanrooij S, Suomalainen A, Spelbrink JN.. 2009. Twinkle mutations associated with autosomal dominant progressive external ophthalmoplegia lead to impaired helicase function and in vivo mtDNA replication stalling. HUMAN MOLECULAR GENETICS 18 2: 328-40. 2009
• Goffart S, Spelbrink H. 2009. Inducible expression in human cells, purification, and in vitro assays for the mitochondrial DNA helicase Twinkle. Methods in Molecular Biology 554: 103-19. 2009
• Pohjoismäki JL, Goffart S, Tyynismaa H, Willcox S, Ide T, Kang D, Suomalainen A, Karhunen PJ, Griffith JD, Holt IJ, Jacobs HT. 2009. Human heart mitochondrial DNA is organized in complex catenated networks containing abundant four-way junctions and replication forks. JOURNAL OF BIOLOGICAL CHEMISTRY 284 32: 21446-57. 2009
• Hakonen AH, Goffart S, Marjavaara S, Paetau A, Cooper H, Mattila K, Lampinen M, Sajantila A, Lönnqvist T, Spelbrink JN, Suomalainen A. 2008. Infantile-onset spinocerebellar ataxia and mitochondrial recessive ataxia syndrome are associated with neuronal complex I defect and mtDNA depletion. HUMAN MOLECULAR GENETICS 17 23: 3822-35. 2008
• Franko A, Mayer S, Thiel G, Mercy L, Arnould T, Hornig-Do HT, Wiesner RJ, Goffart S. 2008. CREB-1alpha is recruited to and mediates upregulation of the cytochrome c promoter during enhanced mitochondrial biogenesis accompanying skeletal muscle differentiation. MOLECULAR AND CELLULAR BIOLOGY 28 7: 2446-59. 2008
• Goffart S, Martinsson P, Malka F, Rojo M, Spelbrink JN.. 2007. The mitochondria of cultured mammalian cells: II. Expression and visualization of exogenous proteins in fixed and live cells. Methods in Molecular Biology 372: 17-32. 2007
• Malka F, Auré K, Goffart S, Spelbrink JN, Rojo M.. 2007. The mitochondria of cultured mammalian cells: I. Analysis by immunofluorescence microscopy, histochemistry, subcellular fractionation, and cell fusion. Methods in Molecular Biology 372: 3-16. 2007
• Wanrooij S, Goffart S, Pohjoismäki JL, Yasukawa T, Spelbrink JN.. 2007. Expression of catalytic mutants of the mtDNA helicase Twinkle and polymerase POLG causes distinct replication stalling phenotypes. NUCLEIC ACIDS RESEARCH 35 10: 3238-51. 2007
• Goffart S, Franko A, Clemen CS, Wiesner RJ.. 2006. Alpha-actinin 4 and BAT1 interaction with the cytochrome c promoter upon skeletal muscle differentiation. Current Genetics 49 2: 125-35. 2006
• Pohjoismäki JL, Wanrooij S, Hyvärinen AK, Goffart S, Holt IJ, Spelbrink JN, Jacobs HT. 2006. Alterations to the expression level of mitochondrial transcription factor A, TFAM, modify the mode of mitochondrial DNA replication in cultured human cells.. NUCLEIC ACIDS RESEARCH 34 20: 5815-28. 2006
• Goffart S, von Kleist-Retzow JC, Wiesner RJ.. 2004. Regulation of mitochondrial proliferation in the heart: power-plant failure contributes to cardiac failure in hypertrophy. CARDIOVASCULAR RESEARCH 64 2: 198-207. 2004
• Maniura-Weber K, Goffart S, Garstka HL, Montoya J, Wiesner RJ.. 2004. 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. NUCLEIC ACIDS RESEARCH 32 20: 6015-27. 2004
• Goffart S, Wiesner RJ.. 2003. Regulation and co-ordination of nuclear gene expression during mitochondrial biogenesis. EXPERIMENTAL PHYSIOLOGY 88 1: 33-40. 2003
• Neu R, Goffart S, Wolf K, Schäfer B. 1998. Relocation of urf a from the mitochondrion to the nucleus cures the mitochondrial mutator phenotype in the fission yeast Schizosaccharomyces pombe. MOLECULAR AND GENERAL GENETICS (BERLIN) 258 4: 389-96. 1998