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Publication Detail
Mice carrying ALS mutant TDP-43, but not mutant FUS, display in vivo defects in axonal transport of signalling endosomes
  • Publication Type:
    Journal article
  • Publication Sub Type:
    Article
  • Authors:
    Sleigh J, Tosolini A, Gordon D, Devoy A, Fratta P, Fisher E, Talbot K, Schiavo G
  • Publisher:
    Elsevier
  • Publication date:
    17/03/2020
  • Journal:
    Cell Reports
  • Status:
    Published
  • Print ISSN:
    2211-1247
Abstract
Amyotrophic lateral sclerosis (ALS) is a fatal, progressive neurodegenerative disease resulting from a complex interplay between genetics and environment. Impairments in axonal transport have been identified in several ALS models, but in vivo evidence remains limited, thus their pathogenic importance remains to be fully resolved. We therefore analysed the in vivo dynamics of retrogradely transported, neurotrophin-containing signalling endosomes in nerve axons of two new ALS mouse models with mutations in the RNA processing genes TARDBP and FUS. TDP-43M337V mice, which show neuromuscular pathology without motor neuron loss, displayed axonal transport perturbations manifesting between 1.5 and 3 months and preceding symptom onset. Contrastingly, despite 20% motor neuron loss, transport remained largely unaffected in FusĪ”14/+ mice. Deficiencies in retrograde axonal transport of signalling endosomes are therefore not shared by all ALS-linked genes, indicating that there are mechanistic distinctions in the pathogenesis of ALS caused by mutations in different RNA processing genes.
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Department of Neuromuscular Diseases
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Department of Neuromuscular Diseases
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Department of Neuromuscular Diseases
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Department of Neuromuscular Diseases
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Department of Neuromuscular Diseases
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