Article
In vitro supplementation with deoxynucleoside monophosphates rescues mitochondrial DNA depletion.
Molecular genetics and metabolism - 1 Sept 2012
Bulst Stefanie, Holinski-Feder Elke, Payne Brendan, Abicht Angela, Krause Sabine, Lochmüller Hanns, Chinnery Patrick F, Walter Maggie C, Horvath Rita
Abstract excerpt
Mitochondrial DNA depletion syndromes are a genetically heterogeneous group of often severe diseases, characterized by reduced cellular mitochondrial DNA content. Investigation of potential therapeutic strategies for mitochondrial DNA depletion syndromes will be dependent on good model systems. We have previously suggested that myotubes may be the optimal model system for such studies. Here we firstly validate...
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