Impaired proteasome activity and accumulation of ubiquitinated substrates in a hereditary neuropathy model.

@article{Fortun2005ImpairedPA,
  title={Impaired proteasome activity and accumulation of ubiquitinated substrates in a hereditary neuropathy model.},
  author={Jenny Fortun and Jie Li and Jocelyn C Go and Ali G. Fenstermaker and Bradley S. Fletcher and Lucia Notterpek},
  journal={Journal of neurochemistry},
  year={2005},
  volume={92 6},
  pages={1531-41}
}
Accumulation of misfolded proteins and alterations in the ubiquitin-proteasome pathway are associated with various neurodegenerative conditions of the CNS and PNS. Aggregates containing ubiquitin and peripheral myelin protein 22 (PMP22) have been observed in the Trembler J mouse model of Charcot-Marie-Tooth disease type 1A demyelinating neuropathy. In these nerves, the turnover rate of the newly synthesized PMP22 is reduced, suggesting proteasome impairment. Here we show evidence of proteasome… CONTINUE READING

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