The Loss of TBK1 Kinase Activity in Motor Neurons or in All Cell Types Differentially Impacts ALS Disease Progression in SOD1 Mice

Valeria Gerbino, Esther Kaunga, Junqiang Ye, Daniele Canzio, Sean O'Keeffe, Noam D. Rudnick, Paolo Guarnieri, Cathleen M. Lutz, Tom Maniatis

Research output: Contribution to journalArticlepeer-review

Abstract

DNA sequence variants in the TBK1 gene associate with familial and sporadic ALS. Gerbino et al. show that partial or complete loss-of-function TBK1 mutations alone do not induce neurodegeneration in mice. However, they profoundly affect disease onset and progression in the SOD1 ALS mouse model.

Original languageEnglish (US)
Pages (from-to)789-805.e5
JournalNeuron
Volume106
Issue number5
DOIs
StatePublished - Jun 3 2020
Externally publishedYes

Keywords

  • ALS
  • Amyotrophic Lateral Sclerosis
  • glia
  • interferon response
  • motor neuron autophagy
  • neuroinflammation
  • selective autophagy
  • SOD1 mouse model
  • TBK1

ASJC Scopus subject areas

  • General Neuroscience

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