Messer A, Plummer J
Wadsworth Center for Laboratories and Research, New York State Department of Health, Albany 12201-0509.
Neuromuscul Disord. 1993 Mar;3(2):129-34. doi: 10.1016/0960-8966(93)90004-4.
The mouse mutant Motor neuron degeneration (Mnd) displays an adult-onset progressive degeneration of upper and lower motor neurons, with mild symptoms recognizable at 6 months, leading to spastic paralysis and premature death at 10-12 months on the C57B1/6 background. Despite this late onset, abnormally-accumulating autofluorescent material can be seen in both the spinal cord and other regions as early as the first month. This pigmented material is present in both increasing numbers of cells, and in increasing amounts within individual cells, as the animals age. Motor neurons then go on to degenerate, while most other cell types stabilize. The level of pathological involvement, well before the onset of clear clinical symptoms, suggests that the full degenerative process is an extremely gradual and protracted one with some selectivity for motor neurons.
小鼠突变体运动神经元变性（Mnd）表现出成年期起病的上下运动神经元进行性变性，在6个月时可识别出轻微症状，在C57B1/6背景下，10 - 12个月时会导致痉挛性麻痹和过早死亡。尽管发病较晚，但早在第一个月时，脊髓和其他区域就可见异常积聚的自发荧光物质。随着动物年龄增长，这种色素沉着物质在越来越多的细胞中出现，且单个细胞内的含量也在增加。随后运动神经元开始变性，而大多数其他细胞类型则保持稳定。在明显临床症状出现之前的病理受累程度表明，整个变性过程是一个极其缓慢且持久的过程，对运动神经元具有一定的选择性。
