Altered excitability of motor neurons in a transgenic mouse model of familial amyotrophic lateral sclerosis

Massimo Pieri, Federica Albo, Chiara Gaetti, Alida Spalloni, C. Peter Bengtson, Patrizia Longone, Silvio Cavalcanti, Cristina Zona

Research output: Contribution to journalArticle

Abstract

Various evidence suggests that amyotrophic lateral sclerosis (ALS) selectively affects motor neuron functioning, but electrophysiological alterations of single motor neurons in ALS remains to be documented. In the present work, the excitability of motor neurons has been tested in a transgenic mouse model of a familial form of ALS, associated with a mutation in Cu,Zn superoxide dismutase (Gly93→Ala). Patch-clamp recordings of membrane potential in transgenic mice motor neurons showed that they fire with increased frequency and shorter duration compared to motor neurons from control mice. The passive membrane properties of these neurons were equivalent however. Such results suggest that an altered motor neuron excitability accompanies an ALS associated mutation and that may contribute to the pathogenesis of the disease.

Original languageEnglish
Pages (from-to)153-156
Number of pages4
JournalNeuroscience Letters
Volume351
Issue number3
DOIs
Publication statusPublished - Nov 20 2003

Keywords

  • Action potentials
  • Cellular culture
  • Excitability
  • G93A mice
  • Motor neurons
  • Patch-clamp
  • Superoxide dismutase

ASJC Scopus subject areas

  • Neuroscience(all)

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