@article {1198, title = {Absence of tumor necrosis factor-alpha does not affect motor neuron disease caused by superoxide dismutase 1 mutations.}, journal = {J Neurosci}, volume = {26}, year = {2006}, month = {2006 Nov 1}, pages = {11397-402}, abstract = {

An increase in the expression of the proinflammatory cytokine tumor necrosis factor alpha (TNF-alpha) has been observed in patients with amyotrophic lateral sclerosis (ALS) and in the mice models of the disease. TNF-alpha is a potent activator of macrophages and microglia and, under certain conditions, can induce or exacerbate neuronal cell death. Here, we assessed the contribution of TNF-alpha in motor neuron disease in mice overexpressing mutant superoxide dismutase 1 (SOD1) genes linked to familial ALS. This was accomplished by the generation of mice expressing SOD1(G37R) or SOD1(G93A) mutants in the context of TNF-alpha gene knock out. Surprisingly, the absence of TNF-alpha did not affect the lifespan or the extent of motor neuron loss in SOD1 transgenic mice. These results provide compelling evidence indicating that TNF-alpha does not directly contribute to motor neuron degeneration caused by SOD1 mutations.

}, keywords = {Animals, Mice, Mice, Inbred C57BL, Mice, Knockout, Mice, Transgenic, Motor Neuron Disease, Mutation, Superoxide Dismutase, Tumor Necrosis Factor-alpha}, issn = {1529-2401}, doi = {10.1523/JNEUROSCI.0602-06.2006}, author = {Gowing, Genevi{\`e}ve and Dequen, Florence and Soucy, Genevi{\`e}ve and J-P Julien} }