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

被引:94
作者
Nishie, M
Mori, F
Fujiwara, H
Hasegawa, M
Yoshimoto, M
Iwatsubo, T
Takahashi, H
Wakabayashi, K
机构
[1] Hirosaki Univ, Sch Med, Inst Brain Sci, Dept Neuropathol, Hirosaki, Aomori 0368562, Japan
[2] Univ Tokyo, Grad Sch Pharmaceut Sci, Dept Neuropathol & Neurosci, Tokyo, Japan
[3] Tokyo Metropolitan Org Med Res, Tokyo Inst Psychiat, Dept Mol Neurobiol, Tokyo, Japan
[4] Taisho Pharmaceut, Med Res Labs, Mol Biol Lab, Saitama, Japan
[5] Niigata Univ, Brain Res Inst, Dept Pathol, Niigata, Japan
关键词
multiple system atrophy; alpha-synucleinopathy; neuronal cytoplasmic inclusions; neuropil threads; phosphorylation;
D O I
10.1007/s00401-003-0811-1
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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.
引用
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页码:292 / 298
页数:7
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