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Concluding remarks: The mitochondrial DNA replication bubble has not burst

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Cited by (34)

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    Injection of the TFAM to cell is one of the novel therapeutic approaches which was used for treatment of mitochondrial diseases in human (Iyer 2013; Thomas et al. 2012). Some studies have shown that human cells lacking mtDNA, express TFAM mRNA but lack TFAM protein (Alam et al. 2003; Antoshechkin et al., 1995; Bogenhagen and Clayton, 2003a, b; Bogenhagen et al. 2003c; Bowmaker et al. 2003; Clayton 1991; Diffley et al., 1992; Ekstrand et al. 2004; Garrido et al. 2003; Gensler et al. 2001; Holt et al., 2003; Holt et al. 2000; Larsson et al. 1994; Parisi et al. 1993; Takamatsu et al. 2002). Also showing that over-expression of TFAM reduce mtDNA-encoded gene expression (Lodeiro et al. 2012).

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    2012, Biochimica et Biophysica Acta - Gene Regulatory Mechanisms
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    MtDNA is replicated by an assembly of proteins in a replisome consisting of DNA polymerase γ (pol γ), the mitochondrial single-stranded DNA binding protein (mtSSB), mitochondrial DNA helicase, topoisomerases and RNaseH activities (Table 1 and Fig. 1). Two modes of DNA replication have been proposed to copy the mitochondrial genome, an asynchronous strand displacement model and a strand-coupled bidirectional replication model [2–5]. In the asynchronous strand displacement model, mtDNA is replicated in an asymmetric fashion where DNA synthesis is primed by transcription through the H-strand origin within the D-loop [6].

  • Single deletions in mitochondrial DNA - Molecular mechanisms and disease phenotypes in clinical practice

    2012, Neuromuscular Disorders
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    Unlike nuclear DNA which replicates once during cell division, mtDNA replicates continuously throughout the cell cycle in dividing and post-mitotic cells. Although the replication apparatus is relatively well characterised, the precise mode of mtDNA replication has been intensely debated [24–26]. For many years mtDNA replication was thought to occur exclusively by a strand-displacement model, where a leading strand is synthesised from OH and a lagging strand from the origin of L-strand replication (OL) in an asymmetric and unidirectional pattern [27–30].

  • The human mitochondrial replication fork in health and disease

    2010, Biochimica et Biophysica Acta - Bioenergetics
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