Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression

@article{Wang1999DilatedCA,
  title={Dilated cardiomyopathy and atrioventricular conduction blocks induced by heart-specific inactivation of mitochondrial DNA gene expression},
  author={Jianming Wang and Hans Wilhelmsson and Christian Graff and Hong Li and Anders Oldfors and Pierre Rustin and Jens C. Br{\"u}ning and C Ronald Kahn and D. A. Clayton and Gregory S Barsh and Peter Thor{\'e}n and Nils-G{\"o}ran Larsson},
  journal={Nature Genetics},
  year={1999},
  volume={21},
  pages={133-137}
}
Mutations of mitochondrial DNA (mtDNA) cause several well-recognized human genetic syndromes with deficient oxidative phosphorylation and may also have a role in ageing and acquired diseases of old age. We report here that hallmarks of mtDNA mutation disorders can be reproduced in the mouse using a conditional mutation strategy to manipulate the expression of the gene encoding mitochondrial transcription factor A (Tfam, previously named mtTFA), which regulates transcription and replication of… CONTINUE READING
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