Messer A, Flaherty L
J Neurogenet. 1986 Nov;3(6):345-55. doi: 10.3109/01677068609106858.
A late-onset neurological disease has been identified in a substrain of C57Bl/6 mice. The disorder is characterized by hindlimb weakness and ataxia starting at 5-11 months of age, progressing to severe spastic paralysis of all limbs, with premature death. Histopathology reveals degeneration of upper and lower motoneurons. Both sexes are affected; the mice are fertile, although breeding efficiency is reduced. In outcrosses to wild-type, symptoms have been observed in all obligate heterozygotes, with a similar age range for onset to that of homozygotes. We have designated this autosomal dominant disorder Motor neuron degeneration (Mnd).
在C57Bl/6小鼠的一个亚系中发现了一种迟发性神经疾病。该病症的特征是5至11月龄时出现后肢无力和共济失调，逐渐发展为四肢严重痉挛性麻痹，并伴有过早死亡。组织病理学显示上下运动神经元变性。两性均受影响；小鼠具有生育能力，尽管繁殖效率有所降低。与野生型杂交时，在所有必然杂合子中均观察到症状，发病年龄范围与纯合子相似。我们将这种常染色体显性疾病命名为运动神经元变性（Mnd）。
