Accumulation of Wildtype and ALS-Linked Mutated VAPB Impairs Activity of the Proteasome

@inproceedings{Moumen2011AccumulationOW,
  title={Accumulation of Wildtype and ALS-Linked Mutated VAPB Impairs Activity of the Proteasome},
  author={Anice Moumen and Isabelle Virard and C{\'e}dric Raoul},
  booktitle={PloS one},
  year={2011}
}
Cellular homeostasis relies on a tight control of protein synthesis, folding and degradation, in which the endoplasmic reticulum (ER) quality control and the ubiquitin proteasome system (UPS) have an instrumental function. ER stress and aberrant accumulation of misfolded proteins represent a pathological signature of amyotrophic lateral sclerosis (ALS), a fatal paralytic disorder caused by the selective degeneration of motoneurons in the brain and spinal cord. Mutations in the ER-resident… CONTINUE READING
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