Collard J F, Julien J P
Centre for Research in Neurosciences, McGill University, Montreal, Quebec, Canada.
J Psychiatry Neurosci. 1995 Jan;20(1):80-2.
We reported recently that transgenic mice overexpressing human neurofilament heavy (NF-H) proteins develop a progressive neurological disorder with pathological features resembling those found in amyotrophic lateral sclerosis (ALS) (Côté et al 1993). A simple behavioral test, the grasping ability, has been used here for evaluating the motor dysfunction during aging of NF-H transgenic mice. Transgenic mice overexpressing NF-H proteins are normal at birth but they progressively fail to uphold their weight when tested for their grasping ability. The deficits in motor function during aging correlate with a progressive disruption of peripheral nerve function as evidenced by the atrophy and degeneration of distal axons.
我们最近报道，过度表达人神经丝重链（NF-H）蛋白的转基因小鼠会出现一种进行性神经疾病，其病理特征类似于肌萎缩侧索硬化症（ALS）中发现的病理特征（Côté等人，1993年）。这里使用了一种简单的行为测试，即抓握能力测试，来评估NF-H转基因小鼠衰老过程中的运动功能障碍。过度表达NF-H蛋白的转基因小鼠出生时正常，但在测试其抓握能力时，它们逐渐无法支撑自身重量。衰老过程中运动功能的缺陷与周围神经功能的逐渐破坏相关，这可通过远端轴突的萎缩和退化得到证明。
