A systematic study of brainstem motor nuclei in a mouse model of ALS, the effects of lithium

Michela Ferrucci, Alida Spalloni, Alessia Bartalucci, Emanuela Cantafora, Federica Fulceri, Michele Nutini, Patrizia Longone, Antonio Paparelli, Francesco Fornai

Research output: Contribution to journalArticlepeer-review

Abstract

Transgenic mice expressing the human superoxide dismutase 1 (SOD-1) mutant at position 93 (G93A) develop a phenotype resembling amyotrophic lateral sclerosis (ALS). In fact, G93A mice develop progressive motor deficits which finally lead to motor palsy and death. This is due to the progressive degeneration of motor neurons in the ventral horn of the spinal cord. Although a similar loss is reported for specific cranial motor nuclei, only a few studies so far investigated degeneration in a few brainstem nuclei. We recently reported that chronic lithium administration delays onset and duration of the disease, while reducing degeneration of spinal motor neuron. In the present study, we extended this investigation to all somatic motor nuclei of the brain stem in the G93A mice and we evaluated whether analogous protective effects induced by lithium in the spinal cord were present at the brain stem level. We found that all motor but the oculomotor nuclei were markedly degenerated in G93A mice, and chronic treatment with lithium significantly attenuated neurodegeneration in the trigeminal, facial, ambiguus, and hypoglossal nuclei. Moreover, in the hypoglossal nucleus, we found that recurrent collaterals were markedly lost in G93A mice while they were rescued by chronic lithium administration.

Original languageEnglish
Pages (from-to)370-383
Number of pages14
JournalNeurobiology of Disease
Volume37
Issue number2
DOIs
Publication statusPublished - Feb 2010

Keywords

  • Amyotrophic lateral sclerosis
  • Brainstem motor neurons
  • Choline acetyl transferase
  • Lithium
  • Synaptic boutons
  • Transgenic G93A mouse

ASJC Scopus subject areas

  • Neurology

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