Sobell Department of Motor Neuroscience and Movement Disorders, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK.
Muscle Nerve. 2010 Jun;41(6):774-84. doi: 10.1002/mus.21579.
Non-invasive excitability studies of motor axons in patients with amyotrophic lateral sclerosis (ALS) have revealed a changing pattern of abnormal membrane properties with disease progression, but the heterogeneity of the changes has made it difficult to relate them to pathophysiology. The SOD1(G93A) mouse model of ALS displays more synchronous motoneuron pathology. Multiple excitability measures of caudal and sciatic nerves in mutant and wild-type mice were compared before onset of signs and during disease progression (4-19 weeks), and they were related to changes in muscle fiber histochemistry. Excitability differences indicated a modest membrane depolarization in SOD1(G93A) axons at about the time of symptom onset (8 weeks), possibly due to deficient energy supply. Previously described excitability changes in ALS patients, suggesting altered sodium and potassium conductances, were not seen in the mice. This suggests that those changes relate to features of the human disease that are not well represented in the animal model.
对肌萎缩侧索硬化症（ALS）患者运动轴突的非侵入性兴奋性研究显示，随着疾病的进展，异常膜特性的模式发生变化，但变化的异质性使得难以将其与病理生理学联系起来。SOD1（G93A）ALS 小鼠模型显示出更同步的运动神经元病理学。在出现症状前和疾病进展期间（4-19 周）比较了突变型和野生型小鼠的尾部和坐骨神经的多种兴奋性测量值，并将其与肌肉纤维组织化学变化相关联。兴奋性差异表明，在症状出现时（8 周），SOD1（G93A）轴突中的膜轻微去极化，这可能是由于能量供应不足所致。以前在 ALS 患者中描述的兴奋性变化表明钠离子和钾离子传导能力改变，但在小鼠中并未观察到。这表明这些变化与人类疾病的特征有关，而这些特征在动物模型中没有很好地体现。
