Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia

@article{Atorino2003LossOM,
  title={Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia},
  author={Luigia Atorino and Laura Silvestri and Mirko Koppen and Laura Cassina and Andrea Ballabio and R Marconi and Thomas Langer and Giorgio Casari},
  journal={The Journal of Cell Biology},
  year={2003},
  volume={163},
  pages={777 - 787}
}
Mmutations in paraplegin, a putative mitochondrial metallopeptidase of the AAA family, cause an autosomal recessive form of hereditary spastic paraplegia (HSP). Here, we analyze the function of paraplegin at the cellular level and characterize the phenotypic defects of HSP patients' cells lacking this protein. We demonstrate that paraplegin coassembles with a homologous protein, AFG3L2, in the mitochondrial inner membrane. These two proteins form a high molecular mass complex, which we show to… CONTINUE READING