Ono S, Imai T, Munakata S, Takahashi K, Kanda F, Hashimoto K, Yamano T, Shimizu N, Nagao K, Yamauchi M
Department of Neurology, Teikyo University School of Medicine, Ichihara Hospital, Anesaki, Chiba, Japan.
J Neurol Sci. 1998 Oct 8;160(2):140-7. doi: 10.1016/s0022-510x(98)00223-8.
During the last 10 years, we have demonstrated morphological and biochemical abnormalities of skin extracellular matrices in amyotrophic lateral sclerosis (ALS). However, currently little is known concerning collagen of the spinal cord in ALS. We measured the amount of collagen and characterized collagen at light and electron microscopic levels in posterior funiculus, posterior half of lateral funiculus and anterior horn of cervical enlargement of the spinal cord obtained from ten patients with ALS, 11 patients with other neurologic diseases (control group A), and ten patients without neurologic ones (control group B). In posterior half of lateral funiculus and anterior horn, (1) by light microscopy, there was no significant difference in vessel wall area between ALS patients and control groups A and B; (2) ultrastructurally, collagen bundles were more fragmented and widely separated, and the fibrils were randomly oriented in the perivascular space of capillaries in ALS patients, which were not observed in any areas of control groups or in posterior funiculus of ALS patients; and (3) the collagen contents in ALS were significantly lower (P<0.001 and P<0.001, respectively) than those in control groups A and B. Fragmented and widely separated collagen bundles in the interstitial tissue surrounding capillaries and markedly decreased amount of collagen in posterior half of lateral funiculus and in anterior horn of ALS could be related to the degeneration of the upper and lower motor neurons in the spinal cord in ALS, that is, selective neuronal vulnerability in ALS.
在过去的10年里，我们已经证明了肌萎缩侧索硬化症（ALS）患者皮肤细胞外基质存在形态学和生化异常。然而，目前对于ALS患者脊髓中的胶原蛋白了解甚少。我们测量了从10例ALS患者、11例患有其他神经系统疾病的患者（对照组A）和10例无神经系统疾病的患者（对照组B）获取的脊髓颈膨大后索、外侧索后半部和前角中胶原蛋白的含量，并在光镜和电镜水平对胶原蛋白进行了表征。在外侧索后半部和前角，（1）光镜下，ALS患者与对照组A和B之间的血管壁面积无显著差异；（2）超微结构上，ALS患者毛细血管周围血管间隙中的胶原束更破碎且间距更宽，原纤维随机排列，而对照组任何区域或ALS患者后索均未观察到这种情况；（3）ALS患者的胶原蛋白含量显著低于对照组A和B（分别为P<0.001和P<0.001）。ALS患者外侧索后半部和前角中毛细血管周围间质组织中破碎且间距宽的胶原束以及胶原蛋白量的显著减少可能与ALS患者脊髓上下运动神经元的变性有关，即ALS中的选择性神经元易损性。
