Leigh P N, Dodson A, Swash M, Brion J P, Anderton B H
Department of Medicine 1, St George's Hospital Medical School, London, UK.
Brain. 1989 Apr;112 ( Pt 2):521-35. doi: 10.1093/brain/112.2.521.
Immunocytochemistry with antibodies against cytoskeletal proteins has been used to search for molecular differences in the spinal cord from patients with motor neuron disease (MND) of amyotrophic lateral sclerosis type and normal spinal cord. Monoclonal antibodies which recognize phosphorylated neurofilament epitopes diffusely labelled a proportion of normal and MND anterior horn cells, but did not permit differentiation between normal and MND tissue. However, in some MND and control anterior horn cells, dense 'floccular' accumulations were labelled by antibodies recognizing phosphorylated neurofilament epitopes. These accumulations of phosphorylated neurofilaments suggest abnormalities of cytoskeletal regulation, but were neither a common nor a specific feature of MND. Axonal spheroids, which were as common in normal as in MND tissue, were labelled by all antineurofilament antibodies. Normal-appearing axons, but not spheroids, in MND and control tissue were identified by an antiactin antibody, indicating that actin may be absent from the cytoplasmic domain which gives rise to spheroids. In summary, we have not found specific posttranslational changes of cytoskeletal proteins in MND and, in particular, phosphorylated neurofilament epitopes are common to both MND and control anterior horn cells.
利用针对细胞骨架蛋白的抗体进行免疫细胞化学检测，以探寻肌萎缩侧索硬化型运动神经元病（MND）患者与正常脊髓在分子水平上的差异。识别磷酸化神经丝表位的单克隆抗体可弥散标记一部分正常及MND前角细胞，但无法区分正常组织与MND组织。然而，在一些MND及对照前角细胞中，识别磷酸化神经丝表位的抗体标记出了密集的“絮状”聚积物。这些磷酸化神经丝的聚积提示细胞骨架调节存在异常，但并非MND的常见或特异性特征。轴突球状体在正常组织和MND组织中出现的频率相同，所有抗神经丝抗体均可将其标记。抗肌动蛋白抗体可识别MND及对照组织中外观正常的轴突，但无法识别球状体，这表明形成球状体的胞质区域可能缺乏肌动蛋白。总之，我们未在MND中发现细胞骨架蛋白的特异性翻译后变化，尤其是磷酸化神经丝表位在MND和对照前角细胞中均较为常见。
