Group of Neuroplasticity and Regeneration, Institute of Neurosciences and Department of Cell Biology, Physiology and Immunology, Universitat Autònoma de Barcelona, and Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Bellaterra, Spain.
Brain Res. 2011 Aug 11;1406:65-73. doi: 10.1016/j.brainres.2011.06.033. Epub 2011 Jul 5.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by the loss of upper and lower motoneurons. Clinically, it is manifested by weakness, muscle atrophy and progressive paralysis and ends up with patients' death 2-5 years after diagnosis. Although these symptoms lead in many cases to gait deficits in patients, an exhaustive locomotor profile of animal models mimicking the disease has not been assessed yet. In this work we evaluated the locomotor performance of the SOD1(G93A) mouse model of ALS using computerized treadmill gait analysis. SOD1(G93A) mice presented early (8 weeks of age) gait abnormalities, evidenced by an increase in the time of the propulsion phase of hindlimb stance. The alterations progressed during the disease until a complete disturbance of normal gait. This finding is meaningful to the field because the identification of a significant difference in a functional endpoint as early as 8 weeks might be a step forward resolving the debate about treatment of mice prior to the symptomatic phase in efficacy studies. These results also point out that digitizing analysis of treadmill locomotion may be useful to evaluate whether new therapeutic approaches are improving functional outcome of the animals.
肌萎缩侧索硬化症（ALS）是一种神经退行性疾病，其特征是上下运动神经元的丧失。临床上，它表现为虚弱、肌肉萎缩和进行性瘫痪，最终导致患者在诊断后 2-5 年内死亡。尽管这些症状在许多情况下导致患者的步态缺陷，但尚未评估模拟疾病的动物模型的详尽运动学特征。在这项工作中，我们使用计算机化跑步机步态分析评估了 SOD1（G93A）ALS 小鼠模型的运动表现。SOD1（G93A）小鼠在 8 周龄时出现早期步态异常，表现为后肢支撑期推进相时间延长。随着疾病的进展，直到正常步态完全紊乱。这一发现对该领域具有重要意义，因为在 8 周时就确定功能终点的显著差异，可能是在疗效研究中在症状前阶段治疗小鼠的争论中向前迈出的一步。这些结果还表明，跑步机运动的数字化分析可能有助于评估新的治疗方法是否正在改善动物的功能结果。
