Development of a novel therapeutic approach for ALS using anti-TNF antibody

小鼠抗tnf α抗体腹腔注射广泛用于肌萎缩性侧索硬化症动物模型的SOD1小鼠。我们从生理学和病理学上评估了治疗或抑制疾病进展的疗效。在生理评估方面，包括寿命、体重和运动功能，没有显著的结果。然而，病理检查显示部分改善反应，包括存活的运动神经元数量和反应性胶质瘤。这些结果进一步支持了SOD1突变体可能在运动神经元死亡的末期参与神经炎症。评估下游级联，如NF kappa， JNK-AP-1和caspase 3，是优化使用抗tnf α抗体治疗的必要条件。

Mouse anti-TNF alpha antibody was intraperitoneally administered to SOD1 mice, which are widely used for animal model for amyotrophic lateral sclerosis. We physiologically and pathologically evaluated the efficacy of the therapy or suppression of disease progression. No significant result was obtained in physiological evaluations, including life span, body weight and motor functions. However, pathological examination revealed partial ameliorative response, including the numbers of survival motor neurons and the reactive gliosis. These results further supported involvement of neuroinflammation in motor neuron death by mutant SOD1 probably at the end stage. Evaluation of the downstream cascades, such as NF kappa, JNK-AP-1 and caspase 3, is necessary to optimize the therapy using the anti-TNF alpha antibody.