INHIBITION OF TERMINAL AXONAL SPROUTING BY SERUM FROM PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS
INHIBITION OF TERMINAL AXONAL SPROUTING BY SERUM FROM PATIENTS WITH AMYOTROPHIC LATERAL SCLEROSIS
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DOI：
10.1056/nejm198410113111501
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发表时间：
1984-01-01
影响因子：
158.5
通讯作者：
ANTEL, JP
中科院分区：
文献类型：
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作者：
GURNEY, ME;BELTON, AC;ANTEL, JP
为了探讨肌萎缩性侧索硬化症的发病机制，我们比较了肌萎缩性侧索硬化症患者血清与对照组和糖尿病周围神经病变患者血清对肉毒杆菌处理小鼠臀肌末梢轴突再生发芽的影响。19例散发型肌萎缩性侧索硬化症患者中有9例和6例家族型肌萎缩性侧索硬化症患者中有2例的血清与对照组或糖尿病患者血清治疗的肌肉相比，导致末梢轴突发芽比例减少。来自肌萎缩性侧索硬化症患者的Ig在免疫印迹上检测时，识别出由失神经大鼠膈肌分泌的56千道尔顿蛋白；兔抗血清也能抑制末端轴突发芽。在肌萎缩侧索硬化症患者的血清中检测到一种抑制神经元发芽和随后的骨骼肌神经再生的抗体。这种抗体是否具有原发致病的重要性，还是代表神经肌肉破坏的二次反应尚不清楚。肌萎缩性侧索硬化症患者的血清可为肌肉和运动神经元之间的营养通讯研究提供试剂。
To investigate the pathogenesis of amyotrophic lateral sclerosis, the effect of serum from patients with this disease on the regenerative sprouting of terminal axons in botulinum-treated mouse gluteus muscle was compared with the effects of serum from controls and from patients with diabetic peripheral neuropathy. Serum from 9 of 19 patients with the sporadic form of amyotrophic lateral sclerosis and from 2 of 6 patients with the familial form caused a reduction in the proportion of sprouting terminal axons, as compared with that found in muscles treated with serum from controls or diabetic patients. Ig from patients with amyotrophic lateral sclerosis, when tested on immunoblots, recognized a 56-kilodalton protein secreted by denervated rat diaphragm muscle; rabbit antiserum raised against this protein also suppressed terminal axonal sprouting. An antibody in the patients'' serum with amyotrophic lateral sclerosis that inhibits neuron sprouting and subsequent skeletal muscle reinnervation was detected. Whether this antibody is of primary pathogenic importance or represents a secondary response to neuromuscular destruction is not known. Serum from patients with amyotrophic lateral sclerosis may provide reagents for trophic communication studies between muscle and motor neurons.