Department of Medicine, McMaster University, 1200 Main St West, Hamilton, Ontario L8N3Z5, Canada.
BMC Neurosci. 2011 Jul 18;12:71. doi: 10.1186/1471-2202-12-71.
The primary cilium is a solitary organelle important in cellular signaling, that projects from the cell surface of most growth-arrested or post-mitotic cells including neurons in the central nervous system. We hypothesized that primary cilial dysfunction might play a role in the pathogenesis of Amyotrophic Lateral Sclerosis (ALS), and as a first step, report on the prevalence of primary cilial markers on cultured motor neurons from the lumbar spinal cord of embryonic wildtype (WT) and transgenic G93A SOD1 mice, and on motor neurons in situ in the lumbar spinal cord.
At 7 days in culture there is no difference in the proportion of G93A SOD1 and WT motor neurons staining for the cilial marker ACIII. However, at 21 days there is a large relative drop in the proportion of ciliated G93A SOD1 motor neurons. In situ, at 40 days there was a slight relative drop in the proportion of ciliated motor neurons in G93A SOD1 mice. At 98 days of age there was no change in motor neuron ciliation in WT mice, but there was motor neuron loss and a large reduction in the proportion of surviving motor neurons bearing a primary cilium in G93A SOD1 mice.
In primary culture and in situ in G93A SOD1 mice there is a large reduction in the proportion of motor neurons bearing a primary cilium.
初级纤毛是一种重要的细胞信号转导细胞器，存在于大多数静止期或有丝分裂后细胞的细胞表面，包括中枢神经系统中的神经元。我们假设初级纤毛功能障碍可能在肌萎缩侧索硬化症（ALS）的发病机制中发挥作用，作为第一步，报告来自胚胎野生型（WT）和转 G93A SOD1 小鼠的腰椎脊髓培养运动神经元以及原位腰椎脊髓运动神经元的初级纤毛标记物的患病率。
在培养的第 7 天，G93A SOD1 和 WT 运动神经元的纤毛标记物 ACIII 染色的比例没有差异。然而，在第 21 天，G93A SOD1 运动神经元有纤毛的比例相对大量下降。在原位，在第 40 天，G93A SOD1 小鼠有纤毛的运动神经元比例略有相对下降。在 WT 小鼠中，98 天龄时运动神经元纤毛没有变化，但在 G93A SOD1 小鼠中，运动神经元丢失和有纤毛的存活运动神经元比例大量减少。
在 G93A SOD1 小鼠的原代培养和原位中，有纤毛的运动神经元比例大量减少。
