Article
Overexpression of survival motor neuron improves neuromuscular function and motor neuron survival in mutant SOD1 mice.
Neurobiology of aging - 1 Apr 2014
Turner Bradley J, Alfazema Neza, Sheean Rebecca K, Sleigh James N, Davies Kay E, Horne Malcolm K, Talbot Kevin
Abstract excerpt
Spinal muscular atrophy results from diminished levels of survival motor neuron (SMN) protein in spinal motor neurons. Low levels of SMN also occur in models of amyotrophic lateral sclerosis (ALS) caused by mutant superoxide dismutase 1 (SOD1) and genetic reduction of SMN levels exacerbates the phenotype of transgenic SOD1(G93A) mice. Here, we demonstrate that SMN protein is significantly reduced in the spinal...
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