Article
Abnormal motor phenotype in the SMNDelta7 mouse model of spinal muscular atrophy.
Neurobiology of disease - 1 Aug 2007
Butchbach Matthew E R, Edwards Jonathan D, Burghes Arthur H M
Abstract excerpt
Spinal muscular atrophy (SMA) is a recessive motor neuron disease that affects motor neurons in the anterior horn of the spinal cord. SMA results from the reduction of SMN (survival motor neuron) protein. Even though SMN is ubiquitously expressed, motor neurons are more sensitive to the reduction in SMN than other cell types. We have previously generated mouse models of SMA with varying degrees of clinical...
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