Journal
NEUROSCIENCE LETTERS
Volume 351, Issue 3, Pages 153-156Publisher
ELSEVIER IRELAND LTD
DOI: 10.1016/j.neulet.2003.07.010
Keywords
action potentials; motor neurons; G93A mice; excitability; cellular culture; superoxide dismutase; patch-clamp
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Funding
- Telethon [1185] Funding Source: Medline
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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 (Gly(93) --> 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. (C) 2003 Elsevier Ireland Ltd. All rights reserved.
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