Regulation and co-ordination of nuclear gene expression during mitochondrial biogenesis S Goffart, RJ Wiesner Experimental physiology 88 (1), 33-40, 2003 | 229 | 2003 |
Regulation of mitochondrial proliferation in the heart: power-plant failure contributes to cardiac failure in hypertrophy S Goffart, JC von Kleist-Retzow, RJ Wiesner Cardiovascular research 64 (2), 198-207, 2004 | 219 | 2004 |
Twinkle helicase (PEO1) gene mutation causes mitochondrial DNA depletion E Sarzi, S Goffart, V Serre, D Chrétien, A Slama, A Munnich, JN Spelbrink, ... Annals of Neurology: Official Journal of the American Neurological …, 2007 | 208 | 2007 |
Alterations to the expression level of mitochondrial transcription factor A, TFAM, modify the mode of mitochondrial DNA replication in cultured human cells JLO Pohjoismäki, S Wanrooij, AK Hyvärinen, S Goffart, IJ Holt, ... Nucleic acids research 34 (20), 5815-5828, 2006 | 192 | 2006 |
Transient overexpression of mitochondrial transcription factor A (TFAM) is sufficient to stimulate mitochondrial DNA transcription, but not sufficient to increase mtDNA copy … K Maniura-Weber, S Goffart, HL Garstka, J Montoya, RJ Wiesner Nucleic acids research 32 (20), 6015-6027, 2004 | 179 | 2004 |
Twinkle mutations associated with autosomal dominant progressive external ophthalmoplegia lead to impaired helicase function and in vivo mtDNA … S Goffart, HM Cooper, H Tyynismaa, S Wanrooij, A Suomalainen, ... Human molecular genetics 18 (2), 328-340, 2009 | 159 | 2009 |
Expression of catalytic mutants of the mtDNA helicase Twinkle and polymerase POLG causes distinct replication stalling phenotypes S Wanrooij, S Goffart, JLO Pohjoismäki, T Yasukawa, JN Spelbrink Nucleic acids research 35 (10), 3238-3251, 2007 | 159 | 2007 |
Infantile-onset spinocerebellar ataxia and mitochondrial recessive ataxia syndrome are associated with neuronal complex I defect and mtDNA depletion AH Hakonen, S Goffart, S Marjavaara, A Paetau, H Cooper, K Mattila, ... Human molecular genetics 17 (23), 3822-3835, 2008 | 158 | 2008 |
Mammalian mitochondrial DNA replication intermediates are essentially duplex but contain extensive tracts of RNA/DNA hybrid JLO Pohjoismäki, JB Holmes, SR Wood, MY Yang, T Yasukawa, A Reyes, ... Journal of molecular biology 397 (5), 1144-1155, 2010 | 157 | 2010 |
Human heart mitochondrial DNA is organized in complex catenated networks containing abundant four-way junctions and replication forks JLO Pohjoismäki, S Goffart, H Tyynismaa, S Willcox, T Ide, D Kang, ... Journal of Biological Chemistry 284 (32), 21446-21457, 2009 | 148 | 2009 |
The role of mitochondria in cardiac development and protection JL Pohjoismäki, S Goffart Free Radical Biology and Medicine 106, 345-354, 2017 | 133 | 2017 |
Tissue specific differences in mitochondrial DNA maintenance and expression E Herbers, NJ Kekäläinen, A Hangas, JL Pohjoismäki, S Goffart Mitochondrion 44, 85-92, 2019 | 124 | 2019 |
Ciprofloxacin impairs mitochondrial DNA replication initiation through inhibition of Topoisomerase 2 A Hangas, K Aasumets, NJ Kekäläinen, M Paloheinä, JL Pohjoismäki, ... Nucleic acids research 46 (18), 9625-9636, 2018 | 120 | 2018 |
ATAD3 controls mitochondrial cristae structure in mouse muscle, influencing mtDNA replication and cholesterol levels S Peralta, S Goffart, SL Williams, F Diaz, S Garcia, N Nissanka, ... Journal of cell science 131 (13), jcs217075, 2018 | 101 | 2018 |
Of circles, forks and humanity: Topological organisation and replication of mammalian mitochondrial DNA JLO Pohjoismäki, S Goffart Bioessays 33 (4), 290-299, 2011 | 94 | 2011 |
PrimPol is required for replication reinitiation after mtDNA damage R Torregrosa-Muñumer, JME Forslund, S Goffart, A Pfeiffer, G Stojkovič, ... Proceedings of the National Academy of Sciences 114 (43), 11398-11403, 2017 | 91 | 2017 |
The accessory subunit of mitochondrial DNA polymerase γ determines the DNA content of mitochondrial nucleoids in human cultured cells M Di Re, H Sembongi, J He, A Reyes, T Yasukawa, P Martinsson, ... Nucleic Acids Research 37 (17), 5701-5713, 2009 | 90 | 2009 |
Defects in mtDNA replication challenge nuclear genome stability through nucleotide depletion and provide a unifying mechanism for mouse progerias RH Hämäläinen, JC Landoni, KJ Ahlqvist, S Goffart, S Ryytty, ... Nature Metabolism 1 (10), 958-965, 2019 | 74 | 2019 |
Oxidative stress during mitochondrial biogenesis compromises mtDNA integrity in growing hearts and induces a global DNA repair response JLO Pohjoismäki, T Boettger, Z Liu, S Goffart, M Szibor, T Braun Nucleic acids research 40 (14), 6595-6607, 2012 | 70 | 2012 |
Developmental and pathological changes in the human cardiac muscle mitochondrial DNA organization, replication and copy number JLO Pohjoismäki, S Goffart, RW Taylor, DM Turnbull, A Suomalainen, ... PloS one 5 (5), e10426, 2010 | 63 | 2010 |