Filamentous aggregations of phosphorylated α-synuclein in Schwann cells (Schwann cell cytoplasmic inclusions) in multiple system atrophy.
Filamentous aggregations of phosphorylated α-synuclein in Schwann cells (Schwann cell cytoplasmic inclusions) in multiple system atrophy.
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DOI：
10.1186/s40478-015-0208-0
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发表时间：
2015-05-21
影响因子：
7.1
通讯作者：
Wakabayashi K
中科院分区：
文献类型：
--
作者：
Nakamura K;Mori F;Kon T;Tanji K;Miki Y;Tomiyama M;Kurotaki H;Toyoshima Y;Kakita A;Takahashi H;Yamada M;Wakabayashi K
多系统萎缩（MSA）的组织学标志是少突胶质细胞中存在磷酸化α-突触核蛋白的丝状聚集体，称为胶质细胞胞质包涵体（GCI）。虽然GCI可广泛发生于中枢神经系统，但在MSA中尚未报道磷酸化α-突触核蛋白在雪旺细胞中的蓄积。我们对14例MSA患者和20例对照组的颅神经、脊神经、外周神经节和内脏自主神经系统进行了化学检测。在MSA中，磷酸化的α-synuclein在雪旺细胞的胞质中被发现积聚。这些雪旺细胞胞质内含物（SCCI）也免疫阳性泛素和p62。14例MSA患者中12例（85.7%）发现SCCI。它们最常见于骶髓前神经，其次是脑神经（眼神经、舌咽迷走神经和舌下神经）以及脊神经节和交感神经节。SCCI很少见于内脏器官。免疫电镜显示SCCI由直径为15-20 nm的异常细丝组成。在对照组中未发现此类夹杂物。目前的研究结果表明，雪旺细胞也参与了MSA的疾病过程。
The histological hallmark of multiple system atrophy (MSA) is the presence of filamentous aggregations of phosphorylated α-synuclein in oligodendrocytes, referred to as glial cytoplasmic inclusions (GCIs). Although GCIs can occur widely in the central nervous system, accumulation of phosphorylated α-synuclein in Schwann cells has not been reported in MSA. We immunohistochemically examined the cranial and spinal nerves, peripheral ganglia and visceral autonomic nervous system of patients with MSA (n = 14) and control subjects (n = 20). In MSA, accumulation of phosphorylated α-synuclein was found in the cytoplasm of Schwann cells. These Schwann cell cytoplasmic inclusions (SCCIs) were also immunopositive for ubiquitin and p62. SCCIs were found in 12 of 14 patients with MSA (85.7 %). They were most frequent in the anterior nerve of the sacral cord and, to a lesser extent, in the cranial nerves (oculomotor, glossopharyngeal-vagus and hypoglossal nerves), and spinal and sympathetic ganglia. SCCIs were rarely found in the visceral organs. Immunoelectron microscopy demonstrated that the SCCIs consisted of abnormal filaments, 15–20 nm in diameter. No such inclusions were found in controls. The present findings indicate that Schwann cells are also involved in the disease process of MSA.
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影响因子：
2.3
作者：
Arima, K
通讯作者：
Arima, K
影响因子：
5
作者：
Hishikawa, N;Hashizume, Y;Sobue, G
通讯作者：
Sobue, G
影响因子：
5.3
作者：
Beirowski B
通讯作者：
Beirowski B
DOI：
10.1097/00005072-199009000-00007
发表时间：
1990-09-01
影响因子：
3.2
作者：
NAKAZATO, Y;YAMAZAKI, H;YAMAGUCHI, H
通讯作者：
YAMAGUCHI, H
影响因子：
12.7
作者：
ARIMA, K;MURAYAMA, S;INOSE, T
通讯作者：
INOSE, T