Neuromuscular defects and breathing disorders in a new mouse model of spinal muscular atrophy

Neuromuscular defects and breathing disorders in a new mouse model of spinal muscular atrophy

Spinal muscular atrophy (SMA) is caused by insufficient levels of the survival motor neuron (SMN) protein leading to muscle paralysis and respiratory failure. In mouse, introducing the human SMN2 gene partially rescues Smn-/- embryonic lethality. However current models were either too severe or near...

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Bibliographic Details
Main Authors: Magali Michaud, Thomas Arnoux, Serena Bielli, Estelle Durand, Yann Rotrou, Sibylle Jablonka, Fabrice Robert, Marc Giraudon-Paoli, Markus Riessland, Marie-Geneviève Mattei, Emile Andriambeloson, Brunhilde Wirth, Michael Sendtner, Jorge Gallego, Rebecca M. Pruss, Thierry Bordet
Format: Article
Language:English
Published: Elsevier 2010-04-01
Series:Neurobiology of Disease
Subjects:
Spinal muscular atrophy
Motor neuron disease
Mouse model
Survival motor neuron gene
Neurodegeneration
Behavior
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996110000082

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http://www.sciencedirect.com/science/article/pii/S0969996110000082

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