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.

Pieri, M., Albo, F., Gaetti, C., Spalloni, A., Bengtson, C., Longone, P., et al. (2003). Altered excitability of motor neurons in a transgenic mouse model of familial amyotrophic lateral sclerosis. NEUROSCIENCE LETTERS, 351(3), 153-156 [10.1016/S0304-3940(03)00945-5].

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

PIERI, MASSIMO;ZONA, CRISTINA
2003-11-20

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 (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.
20-nov-2003
Pubblicato
Rilevanza internazionale
Articolo
Sì, ma tipo non specificato
Settore BIO/09 - FISIOLOGIA
English
Con Impact Factor ISI
Superoxide Dismutase; Animals; Amyotrophic Lateral Sclerosis; Humans; Action Potentials; Disease Models, Animal; Mice; Mice, Transgenic; Mutation; Motor Neurons
Pieri, M., Albo, F., Gaetti, C., Spalloni, A., Bengtson, C., Longone, P., et al. (2003). Altered excitability of motor neurons in a transgenic mouse model of familial amyotrophic lateral sclerosis. NEUROSCIENCE LETTERS, 351(3), 153-156 [10.1016/S0304-3940(03)00945-5].
Pieri, M; Albo, F; Gaetti, C; Spalloni, A; Bengtson, C; Longone, P; Cavalcanti, S; Zona, C
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2108/65508
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