Accumulation of phosphorylated alpha-synuclein in the brain and peripheral ganglia of patients with multiple system atrophy.

Accumulation of phosphorylated alpha-synuclein in the brain and peripheral ganglia of patients with multiple system atrophy.
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DOI:
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发表时间:
2004
影响因子:
12.7
通讯作者:
M. Nishie;F. Mori;Hideo Fujiwara;M. Hasegawa;M. Yoshimoto;T. Iwatsubo;H. Takahashi;K. Wakabayashi
M. Nishie;F. Mori;Hideo Fujiwara;M. Hasegawa;M. Yoshimoto;T. Iwatsubo;H. Takahashi;K. Wakabayashi
中科院分区:
医学1区
文献类型:
--
作者:
M. Nishie;F. Mori;Hideo Fujiwara;M. Hasegawa;M. Yoshimoto;T. Iwatsubo;H. Takahashi;K. Wakabayashi

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我们使用磷酸化α -突触核蛋白特异性抗体(抗pser129)对8例多系统萎缩(MSA)患者的大脑和周围交感神经节进行了免疫组织化学检查。磷酸化的α -突触核蛋白沉积在5个细胞位置:少突胶质细胞质和细胞核,以及神经元细胞质、突起和细胞核。在脑桥核和下橄榄核中发现了许多神经元细胞质包涵体(NCIs),在黑质、蓝斑、新皮质和海马神经元中也发现了少量的神经元细胞质包涵体(NCIs)。8例患者中有2例在交感神经节中发现NCIs。此外,抗pser129免疫组化显示广泛的神经病变;脑桥核内可见大量肿胀的神经突,大脑皮层和丘脑较深层可见精致的神经突,基底节区和脑干内可见神经丝状和点状结构。脑桥核和下橄榄核可见弥漫性神经元胞浆染色(前nci)。因此,磷酸化α -突触核蛋白在胶质细胞和神经元细胞中的广泛积累是MSA患者的病理特征。
We immunohistochemically examined the brain and peripheral sympathetic ganglia from eight patients with multiple system atrophy (MSA), using an antibody specific for phosphorylated alpha-synuclein (anti-PSer129). Phosphorylated alpha-synuclein was deposited in five cellular locations: oligodendroglial cytoplasm and nucleus, and neuronal cytoplasm, processes and nucleus. Many neuronal cytoplasmic inclusions (NCIs) were found in the pontine and inferior olivary nuclei and, to a lesser extent, in the substantia nigra, locus ceruleus, and neocortical and hippocampal neurons. NCIs were also found in the sympathetic ganglia in two out of the eight cases. Moreover, anti-PSer129 immunohistochemistry revealed extensive neuropil pathology; swollen neurites were abundant in the pontine nucleus, delicate neurites were observed in the deeper layers of the cerebral cortex and thalamus, and neuropil threads and dot-like structures were distributed in the basal ganglia and brainstem. Diffuse neuronal cytoplasmic staining (pre-NCI) was frequently found in the pontine and inferior olivary nuclei. Thus, the widespread accumulation of phosphorylated alpha-synuclein in both glial and neuronal cells is a pathological feature in patients suffering from MSA.