Abnormalities in whisking behaviour are associated with lesions in brain stem nuclei in a mouse model of amyotrophic lateral sclerosis

Robyn A Grant, Paul S Sharp, Aneurin J Kennerley, Jason Berwick, Andrew Grierson, Tennore Ramesh, Tony J Prescott

Research output: Contribution to journalArticlepeer-review

Abstract

The transgenic SOD1(G93A) mouse is a model of human amyotrophic lateral sclerosis (ALS) and recapitulates many of the pathological hallmarks observed in humans, including motor neuron degeneration in the brain and the spinal cord. In mice, neurodegeneration particularly impacts on the facial nuclei in the brainstem. Motor neurons innervating the whisker pad muscles originate in the facial nucleus of the brain stem, with contractions of these muscles giving rise to "whisking" one of the fastest movements performed by mammals. A longitudinal study was conducted on SOD1(G93A) mice and wild-type litter mate controls, comparing: (i) whisker movements using high-speed video recordings and automated whisker tracking, and (ii) facial nucleus degeneration using MRI. Results indicate that while whisking still occurs in SOD1(G93A) mice and is relatively resistant to neurodegeneration, there are significant disruptions to certain whisking behaviours, which correlate with facial nuclei lesions, and may be as a result of specific facial muscle degeneration. We propose that measures of mouse whisker movement could potentially be used in tandem with measures of limb dysfunction as biomarkers of disease onset and progression in ALS mice and offers a novel method for testing the efficacy of novel therapeutic compounds.

Original languageEnglish
Pages (from-to)274-83
Number of pages10
JournalBehavioural Brain Research
Volume259
Early online date13 Nov 2013
DOIs
Publication statusPublished - 1 Feb 2014

Keywords

  • Age Factors
  • Amyotrophic Lateral Sclerosis
  • Analysis of Variance
  • Animals
  • Brain Stem
  • Disease Models, Animal
  • Disease Progression
  • Electron Transport Complex IV
  • Humans
  • Locomotion
  • Magnetic Resonance Imaging
  • Mice
  • Mice, Transgenic
  • Muscle Strength
  • Superoxide Dismutase
  • Time Factors
  • Vibrissae
  • Journal Article
  • Research Support, Non-U.S. Gov't

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