Downregulation of the human Lon protease impairs mitochondrial structure and function and causes cell death.

@article{Bota2005DownregulationOT,
  title={Downregulation of the human Lon protease impairs mitochondrial structure and function and causes cell death.},
  author={Daniela Annenelie Bota and Jenny K. Ngo and Kelvin J A Davies},
  journal={Free radical biology & medicine},
  year={2005},
  volume={38 5},
  pages={665-77}
}
Lon now emerges as a major regulator of multiple mitochondrial functions in human beings. Lon catalyzes the degradation of oxidatively modified matrix proteins, chaperones the assembly of inner membrane complexes, and participates in the regulation of mitochondrial gene expression and genome integrity. An early result of Lon downregulation in WI-38 VA-13 human lung fibroblasts is massive caspase 3 activation and extensive (although not universal) apoptotic death. At a later stage, the surviving… CONTINUE READING

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