Elliott J L, Snider W D
Department of Neurology, Washington University School of Medicine, St Louis, MO 63110, USA.
Neuroreport. 1995 Feb 15;6(3):449-52. doi: 10.1097/00001756-199502000-00011.
The selective vulnerability of limb and bulbar motor neurons is a hallmark of degenerative human motor neuron diseases such as amyotrophic lateral sclerosis (ALS). Currently, there are no known molecular characteristics to distinguish between motor neuron pools which are highly susceptible to degeneration in ALS and those populations which are resistant. Using in situ hybridization on adult rat tissue, we demonstrated that ALS-resistant motor pools robustly express mRNA for the calcium binding protein parvalbumin, while no measurable parvalbumin expression is found in ALS-sensitive motor neuron populations. In contrast, mRNA expression for each of several other calcium binding proteins such as calbindin-D28K, calretinin and calmodulin appears similar in the various motor pools. Thus, parvalbumin represents a biochemical marker of ALS-resistant motor neurons, and may provide insight into the mechanisms of resistance of certain motor neurons to disease.
肢体和延髓运动神经元的选择性易损性是诸如肌萎缩侧索硬化症（ALS）等退行性人类运动神经元疾病的一个标志。目前，尚无已知的分子特征可区分在ALS中极易发生变性的运动神经元池与具有抗性的运动神经元群体。通过对成年大鼠组织进行原位杂交，我们证明，对ALS具有抗性的运动神经元池强烈表达钙结合蛋白小白蛋白的mRNA，而在对ALS敏感的运动神经元群体中未发现可测量的小白蛋白表达。相比之下，其他几种钙结合蛋白，如钙结合蛋白-D28K、钙视网膜蛋白和钙调蛋白，在各个运动神经元池中的mRNA表达似乎相似。因此，小白蛋白代表了对ALS具有抗性的运动神经元的生化标志物，并可能为某些运动神经元对疾病的抗性机制提供见解。
