ACCUMULATION OF PHOSPHORYLATED NEUROFILAMENTS IN ANTERIOR HORN MOTONEURONS OF AMYOTROPHIC LATERAL SCLEROSIS PATIENTS
ACCUMULATION OF PHOSPHORYLATED NEUROFILAMENTS IN ANTERIOR HORN MOTONEURONS OF AMYOTROPHIC LATERAL SCLEROSIS PATIENTS
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DOI：
10.1097/00005072-198801000-00002
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发表时间：
1988-01-01
影响因子：
3.2
通讯作者：
SELKOE, DJ
中科院分区：
文献类型：
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作者：
MUNOZ, DG;GREENE, C;SELKOE, DJ
在肌萎缩侧索硬化症（ALS）患者的前角运动神经元中已经描述了中间纤维的核周集合，但这些内含物通常被认为是罕见的，主要与ALS的家族形式有关。使用单克隆抗体NF2F11，它识别磷酸化的神经丝表位，我们发现，在前角运动神经元的神经丝的局灶性集合是一个特征性的发现，在散发性和家族性ALS，他们是目前在7个9 ALS患者，但没有在9个控制脊髓。这些神经丝集合体不与针对成对螺旋丝和微管相关蛋白tau的抗体交叉反应。此外，ALS患者前角胞体染色质溶解性磷酸化神经丝抗原决定簇的弥漫染色明显多于对照组。
Perikaryal collections of intermediate filaments have been described in the anterior horn motoneurons of patients with amyotrophic lateral sclerosis (ALS), but these inclusions have generally been considered rare and mainly associated with the familial form of ALS. Using the monoclonal antibody NF2F11, which recognizes phosphorylated neurofilament epitopes, we showed that focal collections of neurofilaments in anterior horn motoneurons were a characteristic finding in sporadic as well as in familial ALS; they were present in seven of nine ALS patients, but in none of nine control spinal cords. These neurofilamentous collections are not cross-reactive with antibodies directed against paired helical filaments anbd the microtubule associated protein tau. In addition, diffuse staining for phosphorylated neurofilament epitopes in chromatolytic anterior horn perikarya was significantly more frequent in ALS patients than in controls.