Late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice.

@article{Srensen2001LateonsetCN,
  title={Late-onset corticohippocampal neurodepletion attributable to catastrophic failure of oxidative phosphorylation in MILON mice.},
  author={Lene S{\"o}rensen and Mats I. Ekstrand and Jose Denison Prado Silva and Eva Lindqvist and Baoji Xu and Pierre Rustin and Lars Olson and N-G. Larsson},
  journal={The Journal of neuroscience : the official journal of the Society for Neuroscience},
  year={2001},
  volume={21 20},
  pages={8082-90}
}
We generated mitochondrial late-onset neurodegeneration (MILON) mice with postnatal disruption of oxidative phosphorylation in forebrain neurons. They develop normally and display no overt behavioral disturbances or histological changes during the first 5 months of life. The MILON mice display reduced levels of mitochondrial DNA and mitochondrial RNA from 2 and 4 months of age, respectively, and severely respiratory chain-deficient neurons from 4 months of age. Surprisingly, these respiratory… CONTINUE READING