Duberley R M, Johnson I P, Anand P, Swash M, Martin J, Leigh P N, Zeman S
Department of Anatomy, Medical Sciences, Queen Mary and Westfield College, London, UK.
J Neurol Sci. 1995 May;129 Suppl:109-13. doi: 10.1016/0022-510x(95)00079-h.
Ciliary neurotrophic factor (CNTF) is known to rescue motor neurones in animal models of injury and neurodegeneration and we have recently described regional changes of CNTF protein levels in spinal cord from patients with sporadic motor neurone disease of the amyotrophic lateral sclerosis (ALS) type. However, information is lacking about the CNTF receptor in this condition. We have therefore studied mRNA levels for the alpha subunit of the CNTF receptor (CNTFR alpha) and for CNTF itself in postmortem spinal cord and cerebral cortex in patients with sporadic ALS and matched controls. We report that in the spinal cord of ALS patients there is a marked increase in the hybridisation signal for CNTFR alpha subunit mRNA overlying motor neurones. In contrast, very little mRNA for CNTFR alpha subunit was found in the motor cortex and no differences were seen between ALS and controls. We were unable to detect any hybridisation signal for CNTF mRNA. Our findings provide evidence for regional differences in CNTF receptor expression in upper- and lower-motor neurones in ALS.
已知睫状神经营养因子（CNTF）可在损伤和神经退行性变的动物模型中挽救运动神经元，并且我们最近描述了肌萎缩侧索硬化症（ALS）型散发性运动神经元病患者脊髓中CNTF蛋白水平的区域变化。然而，关于这种情况下的CNTF受体的信息却很缺乏。因此，我们研究了散发性ALS患者和匹配对照者死后脊髓和大脑皮质中CNTF受体α亚基（CNTFRα）以及CNTF本身的mRNA水平。我们报告，在ALS患者的脊髓中，运动神经元上方的CNTFRα亚基mRNA杂交信号显著增加。相比之下，在运动皮质中发现的CNTFRα亚基mRNA很少，并且在ALS患者和对照者之间未观察到差异。我们未能检测到CNTF mRNA的任何杂交信号。我们的研究结果为ALS患者上下运动神经元中CNTF受体表达的区域差异提供了证据。
