Liu Y, Brooks B R, Taniguchi N, Hartmann H A
Department of Pathology and Laboratory Medicine, University of Wisconsin Medical School 53706, USA.
Acta Neuropathol. 1998 Jan;95(1):63-70. doi: 10.1007/s004010050766.
Motor neurons from the brain stems of amyotrophic lateral sclerosis (ALS) and control patients were examined with immunoantibodies to CuZn-superoxide dismutase (CuZnSOD) and Mn-superoxide dismutase (MnSOD). We found that there was a marked staining for CuZnSOD in all the motor nuclei, the hypoglossus, ambiguus, facialis and trigeminus from the ALS patients, but not in the controls. The same neurons from the ALS patients also stained very intensely for MnSOD, whereas the neurons from the control patients stained weakly or not at all. Loss of neurons was also a very consistent finding and was noted in all the motor nuclei from the ALS patients. There was a proliferation of glial cells which stained strongly both for CuZnSOD and for MnSOD accompanying the loss of the neurons. These results indicated that there was an apparent increase of superoxide dismutase immunoreactivity in motor neurons of ALS patients. We conclude that CuZnSOD and MnSOD immunoreactivity is increased in motor neurons and glia in the brain stems of patients with ALS, specific for the terminal phase of this disease.
使用针对铜锌超氧化物歧化酶（CuZnSOD）和锰超氧化物歧化酶（MnSOD）的免疫抗体，对肌萎缩侧索硬化症（ALS）患者和对照患者脑干中的运动神经元进行了检查。我们发现，ALS患者的所有运动核团、舌下神经核、疑核、面神经核和三叉神经核中，CuZnSOD均有明显染色，而对照患者中则没有。ALS患者的相同神经元对MnSOD也有非常强烈的染色，而对照患者的神经元染色较弱或根本没有染色。神经元丢失也是一个非常一致的发现，在ALS患者的所有运动核团中均有观察到。伴随神经元丢失，胶质细胞出现增殖，这些胶质细胞对CuZnSOD和MnSOD均有强烈染色。这些结果表明，ALS患者运动神经元中超氧化物歧化酶免疫反应性明显增加。我们得出结论，ALS患者脑干中的运动神经元和胶质细胞中CuZnSOD和MnSOD免疫反应性增加，这是该疾病终末期的特异性表现。
