The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy

Spinal muscular atrophy (SMA), which is caused by inactivating mutations in the survival motor neuron 1 (SMN1) gene, is characterized by loss of lower motor neurons in the spinal cord. The gene encoding SMN is very highly conserved in evolution, allowing the disease to be modeled in a range of speci...

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Bibliographic Details
Main Authors: James N. Sleigh, Thomas H. Gillingwater, Kevin Talbot
Format: Article
Language:English
Published: The Company of Biologists 2011-07-01
Series:Disease Models & Mechanisms
Online Access:http://dmm.biologists.org/content/4/4/457