Article
Progressive spinal axonal degeneration and slowness in ALS2-deficient mice.
Annals of neurology - 1 Jul 2006
Yamanaka Koji, Miller Timothy M, McAlonis-Downes Melissa, Chun Seung Joo, Cleveland Don W
Abstract excerpt
OBJECTIVE: Homozygous mutation in the ALS2 gene and the resulting loss of the guanine exchange factor activity of the ALS2 protein is causative for autosomal recessive early-onset motor neuron disease that is thought to predominantly affect upper motor neurons. The goal of this study was to elucidate how the motor system is affected by the deletion of ALS2. METHODS: ALS2-deficient mice were generated by gene...
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