RT Journal Article SR Electronic T1 Absence of Tumor Necrosis Factor-α Does Not Affect Motor Neuron Disease Caused by Superoxide Dismutase 1 Mutations JF The Journal of Neuroscience JO J. Neurosci. FD Society for Neuroscience SP 11397 OP 11402 DO 10.1523/JNEUROSCI.0602-06.2006 VO 26 IS 44 A1 Geneviève Gowing A1 Florence Dequen A1 Geneviève Soucy A1 Jean-Pierre Julien YR 2006 UL http://www.jneurosci.org/content/26/44/11397.abstract AB An increase in the expression of the proinflammatory cytokine tumor necrosis factor α (TNF-α) has been observed in patients with amyotrophic lateral sclerosis (ALS) and in the mice models of the disease. TNF-α 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-α 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 SOD1G37R or SOD1G93A mutants in the context of TNF-α gene knock out. Surprisingly, the absence of TNF-α did not affect the lifespan or the extent of motor neuron loss in SOD1 transgenic mice. These results provide compelling evidence indicating that TNF-α does not directly contribute to motor neuron degeneration caused by SOD1 mutations.