Article
Prediction of death in the SMNΔ7 mouse model of spinal muscular atrophy: insight into disease stage and progression.
Journal of neuroscience methods - 15 Aug 2012
El-Khodor Bassem F, Cirillo Kim, Beltran Jose A, Mushlin Richard, Winberg Margaret L, Charney Rachel, Chomicova Olga, Marino Tara, Ramboz Sylvie
Abstract excerpt
Proximal Spinal Muscular Atrophy (SMA) is a debilitating neuromuscular disease and a leading inherited genetic cause of infant death. To date, there is no effective treatment for SMA. The SMNΔ7 neonatal mouse model of SMA recapitulates key features of the severe form of SMA and remains a valuable tool in preclinical drug discovery. At any particular postnatal age (P), the disease progression in the SMNΔ7 mouse...
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