Scientific article
English

Bassoon proteinopathy drives neurodegeneration in multiple sclerosis

Published inNature Neuroscience, vol. 22, no. 6, p. 887-896
Publication date2019
Abstract

Multiple sclerosis (MS) is characterized by inflammatory insults that drive neuroaxonal injury. However, knowledge about neuron-intrinsic responses to inflammation is limited. By leveraging neuron-specific messenger RNA profiling, we found that neuroinflammation leads to induction and toxic accumulation of the synaptic protein bassoon (Bsn) in the neuronal somata of mice and patients with MS. Neuronal overexpression of Bsn in flies resulted in reduction of lifespan, while genetic disruption of Bsn protected mice from inflammation-induced neuroaxonal injury. Notably, pharmacological proteasome activation boosted the clearance of accumulated Bsn and enhanced neuronal survival. Our study demonstrates that neuroinflammation initiates toxic protein accumulation in neuronal somata and advocates proteasome activation as a potential remedy.

Keywords
  • Animals
  • Drosophila
  • Humans
  • Inflammation/metabolism/pathology
  • Mice
  • Multiple Sclerosis/metabolism/pathology
  • Nerve Degeneration/metabolism/pathology
  • Nerve Tissue Proteins/metabolism
  • Neurons/metabolism/pathology
  • Spinal Cord/metabolism/pathology
Funding
Citation (ISO format)
SCHATTLING, Benjamin et al. Bassoon proteinopathy drives neurodegeneration in multiple sclerosis. In: Nature Neuroscience, 2019, vol. 22, n° 6, p. 887–896. doi: 10.1038/s41593-019-0385-4
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Article (Published version)
accessLevelRestricted
Identifiers
Journal ISSN1097-6256
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