Co-localization ofBunina bodies and TDP-43 inclusionsin lower motor neurons in amyotrophiclateral sclerosis
Co-localization ofBunina bodies and TDP-43 inclusionsin lower motor neurons in amyotrophiclateral sclerosis
复制标题
肌萎缩侧索硬化症下运动神经元布尼纳小体和 TDP-43 包涵体的共定位
DOI：
10.1111/neup.12044
复制
发表时间：
2013
期刊：
影响因子：
2.3
通讯作者：
Takahashi H,Wakabayashi K
中科院分区：
文献类型：
--
作者：
Mori F;Kakita A;Takahashi H,Wakabayashi K
肌萎缩性侧索硬化症（ALS）的特征是运动神经元受累于Bunina小体（BBs）和DNA蛋白43 （TDP‐43）包涵体。我们检查了来自ALS患者的脊髓（n= 20）、舌下核（n= 6）和面核（n= 5），以阐明BBs与TDP - 43内含物之间的关系。20例中有16例在前角，6例在舌下核，5例中有4例在面神经核。在所有病例的每个区域均发现TDP - 43夹杂物。BBs和TDP - 43包涵体共定位于15.2%的前角神经元、29.2%的舌下核和17.3%的面核。在每个区域，有BBs的神经元中TDP - 43包体的频率明显高于没有BBs的神经元。超微结构上，TDP‐43阳性丝状结构与BBs混杂。这些发现表明，BBs与TDP - 43包裹体之间存在密切的关系。
Amyotrophic lateral sclerosis (ALS) is characterized by motor neuron involvement with Bunina bodies (BBs) and transactivation response DNA protein 43 (TDP‐43) inclusions. We examined the spinal cord (n= 20), hypoglossal nucleus (n= 6) and facial nucleus (n= 5) from ALS patients to elucidate the relationship between BBs and TDP‐43 inclusions. BBs were found in the anterior horn in 16 of 20 cases, in the hypoglossal nucleus in all six cases and in the facial nucleus in four out of five cases. TDP‐43 inclusions were found in each region of all the cases. Co‐localization of BBs and TDP‐43 inclusions was found in 15.2% of total neurons in the anterior horn, 29.2% in the hypoglossal nucleus and 17.3% in the facial nucleus. The frequency of TDP‐43 inclusions was significantly higher in neurons with BBs than in those without in each region. Ultrastructurally, TDP‐43‐positive filamentous structures were intermingled with BBs. These findings suggest that there is a close relationship in the occurrence between BBs and TDP‐43 inclusions.