Widespread occurrence of α‐synuclein/NACP‐immunoreactive neuronal inclusions in juvenileand adult‐onset Hallervorden–Spatz diseasewith Lewy bodies
Widespread occurrence of α‐synuclein/NACP‐immunoreactive neuronal inclusions in juvenileand adult‐onset Hallervorden–Spatz diseasewith Lewy bodies
复制标题
青少年和成人发病的路易体 Hallervorden-Spatz 病中广泛存在 α-突触核蛋白/NACP-免疫反应性神经元包涵体
DOI：
--
复制
发表时间：
1999
期刊：
影响因子：
--
通讯作者：
Hoyu Takahashi
中科院分区：
文献类型：
--
作者：
K. Wakabayashi;M. Yoshimoto;T. Fukushima;R. Koide;Y. Horikawa;T. Morita;Hoyu Takahashi
α-突触核蛋白（最初称为阿尔茨海默病淀粉样蛋白-NACP的非Aβ成分的前体）是一种突触前神经末梢蛋白，现在已知是帕金森病中路易体（LB）的主要成分。以前的研究表明，LB偶尔在Hallervorden-Spatz病（HSD）患者中发现，HSD是一种遗传性或散发性神经轴索营养不良。因此，对2例HSD患者的脑组织进行了α-突触核蛋白/NACP免疫细胞化学检查。在这两种情况下，LB观察到黑质，蓝斑和其他皮质下核。这些LB用抗α-突触核蛋白/NACP进行了强免疫标记。此外，在大脑新皮质、海马、基底神经节、丘脑、脑桥和下橄榄核、脊髓灰质和外周交感神经节中发现神经元胞体和突起中的异常α-突触核蛋白/NACP-免疫反应性结构。虽然在整个大脑中发现了许多营养不良的轴突（球状体），但没有或只有少数α-突触核蛋白/NACP呈阳性。这些发现表明，α-突触核蛋白/NACP的广泛积累是患有HSD伴LB的患者的病理特征，并且这种现象与轴突球体形成无关。
α‐Synuclein (originally called precursor of the non‐Aβ component of Alzheimer’s disease amyloid‐NACP) is a presynaptic nerve terminal protein and is now known to be a major component of Lewy bodies (LBs) in Parkinson’s disease. Previous studies have shown that LBs are occasionally found in patients with Hallervorden–Spatz disease (HSD), a hereditary or sporadic neuroaxonal dystrophy. Therefore, an immunocytochemical examination of the brain tissues from two patients with HSD for α‐synuclein/NACP was performed. In both cases, LBs were observed in the substantia nigra, locus ceruleus and other subcortical nuclei. These LBs were strongly immunolabelled with anti‐α‐synuclein/NACP. Moreover, abnormal α‐synuclein/NACP‐immunoreactive structures in the neuronal somata and processes were found in the cerebral neocortex, hippocampus, basal ganglia, thalamus, pontine and inferior olivary nuclei, spinal grey matter, and peripheral sympathetic ganglia. Although numerous dystrophic axons (spheroids) were found throughout the brain, either none or only a few were positive for α‐synuclein/NACP. These findings suggest that widespread accumulation of α‐synuclein/NACP is a pathological feature in patients suffering from HSD with LBs, and that this phenomenon is unrelated to axonal spheroid formation.
DOI：
--
发表时间：
1998-04
期刊：
The American journal of pathology
影响因子：
--
作者：
M. Baba;S. Nakajo;Ping-Hui Tu;T. Tomita;K. Nakaya;V. Lee;J. Trojanowski;T. Iwatsubo
通讯作者：
M. Baba;S. Nakajo;Ping-Hui Tu;T. Tomita;K. Nakaya;V. Lee;J. Trojanowski;T. Iwatsubo
神经退行性疾病中 NACP/α-突触核蛋白的异常积累。
DOI：
--
发表时间：
1998
期刊：
The American journal of pathology
影响因子：
--
作者：
Takeda,A;Mallory,M;Sundsmo,M;Honer,W;Hansen,L;Masliah,E
通讯作者：
Masliah,E