Van der Neut R, Gispen W H, Bär P R
Research Laboratory of Neurology, University Hospital of Utrecht, The Netherlands.
Mol Chem Neuropathol. 1991 Jun;14(3):247-58. doi: 10.1007/BF03159940.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease affecting motor neurons in the spinal cord, brain stem, and cortex. Cultures of fetal rat spinal cord cells were used to test sera from ALS patients (ALS sera) on their ability to influence the expression of the neuron-specific phosphoprotein B-50/GAP-43. Neurons were treated with ALS sera, sera of age-matched controls (CON sera), or sera of patients with autonomic neuropathy (AUTO sera) and fixed after 24 or 96 h. The levels of B-50 and neurofilament (NF) protein were assayed with an enzyme-linked immunoadsorbent assay (ELISA). No toxic effects of the ALS sera were observed. It appeared that after 24 h, both B-50 and NF levels were elevated in the ALS sera-treated cells by 12 and 11%, respectively. After 96 h, the B-50 level was 19% higher than in CON sera-treated neurons, and the NF level was 29% higher. AUTO sera did not differ from CON sera. The stimulating effect of ALS sera was absent if the sera were heated at 56 degrees C for 30 min. We conclude that ALS serum induces the expression of B-50 and the subsequent axonal outgrowth and maturation in vitro. This induction might be a reflection in vitro of the processes underlying the collateral sprouting responses observed in ALS patients.
肌萎缩侧索硬化症（ALS）是一种影响脊髓、脑干和皮层运动神经元的神经退行性疾病。利用胎鼠脊髓细胞培养物来检测肌萎缩侧索硬化症患者血清（ALS血清）影响神经元特异性磷蛋白B-50/GAP-43表达的能力。用ALS血清、年龄匹配对照者的血清（CON血清）或自主神经病变患者的血清（AUTO血清）处理神经元，并在24小时或96小时后固定。用酶联免疫吸附测定（ELISA）检测B-50和神经丝（NF）蛋白的水平。未观察到ALS血清的毒性作用。结果显示，24小时后，ALS血清处理的细胞中B-50和NF水平分别升高了12%和11%。96小时后，B-50水平比CON血清处理的神经元高19%，NF水平高29%。AUTO血清与CON血清无差异。如果将血清在56摄氏度加热30分钟，则ALS血清的刺激作用消失。我们得出结论，ALS血清在体外可诱导B-50的表达以及随后的轴突生长和成熟。这种诱导可能是体外对肌萎缩侧索硬化症患者中观察到的侧支发芽反应潜在过程的一种反映。
