Steffi Goffart

Author PubWeight™ 18.67‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Expression of catalytic mutants of the mtDNA helicase Twinkle and polymerase POLG causes distinct replication stalling phenotypes. Nucleic Acids Res 2007 1.69
2 Twinkle helicase (PEO1) gene mutation causes mitochondrial DNA depletion. Ann Neurol 2007 1.67
3 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 Res 2004 1.53
4 Alterations to the expression level of mitochondrial transcription factor A, TFAM, modify the mode of mitochondrial DNA replication in cultured human cells. Nucleic Acids Res 2006 1.51
5 Mammalian mitochondrial DNA replication intermediates are essentially duplex but contain extensive tracts of RNA/DNA hybrid. J Mol Biol 2010 1.50
6 Infantile-onset spinocerebellar ataxia and mitochondrial recessive ataxia syndrome are associated with neuronal complex I defect and mtDNA depletion. Hum Mol Genet 2008 1.29
7 Human heart mitochondrial DNA is organized in complex catenated networks containing abundant four-way junctions and replication forks. J Biol Chem 2009 1.09
8 Sequence-specific stalling of DNA polymerase γ and the effects of mutations causing progressive ophthalmoplegia. Hum Mol Genet 2011 0.97
9 CREB-1alpha is recruited to and mediates upregulation of the cytochrome c promoter during enhanced mitochondrial biogenesis accompanying skeletal muscle differentiation. Mol Cell Biol 2008 0.94
10 Of circles, forks and humanity: Topological organisation and replication of mammalian mitochondrial DNA. Bioessays 2011 0.91
11 Adaptation of topoisomerase I paralogs to nuclear and mitochondrial DNA. Nucleic Acids Res 2009 0.90
12 Oxidative stress during mitochondrial biogenesis compromises mtDNA integrity in growing hearts and induces a global DNA repair response. Nucleic Acids Res 2012 0.88
13 Developmental and pathological changes in the human cardiac muscle mitochondrial DNA organization, replication and copy number. PLoS One 2010 0.88
14 Overexpression of Twinkle-helicase protects cardiomyocytes from genotoxic stress caused by reactive oxygen species. Proc Natl Acad Sci U S A 2013 0.86
15 Binding to G-quadruplex RNA activates the mitochondrial GTPase NOA1. Biochim Biophys Acta 2013 0.81
16 Replication stalling by catalytically impaired Twinkle induces mitochondrial DNA rearrangements in cultured cells. Mitochondrion 2011 0.80
17 The mitochondria of cultured mammalian cells: I. Analysis by immunofluorescence microscopy, histochemistry, subcellular fractionation, and cell fusion. Methods Mol Biol 2007 0.77