Accumulation of phosphorylated -synuclein in subpial and periventricular astrocytes in multiple system atrophy of long duration

作者：Nakamura Keiko; Mori Fumiaki^*; Kon Tomoya; Tanji Kunikazu; Miki Yasuo; Tomiyama Masahiko; Kurotaki Hidekachi; Toyoshima Yasuko; Kakita Akiyoshi; Takahashi Hitoshi; Yamada Masahito; Wakabayashi Koichi

来源：Neuropathology, 2016, 36(2): 157-167.

DOI：10.1111/neup.12243

• alpha-synuclein
• astrocyte
• multiple system atrophy
• subpial surface
• ultrastructure

The histological hallmark of multiple system atrophy (MSA) is accumulation of phosphorylated alpha-synuclein in oligodendrocytes. However, it is uncertain whether phosphorylated alpha-synuclein accumulates in astrocytes of MSA patients. We immunohistochemically examined the frontal and temporal lobes, basal ganglia, cerebellum, brainstem and spinal cord of patients with MSA (n=15) and Lewy body disease (n=20), and also in control subjects (n=20). Accumulation of abnormally phosphorylated and aggregated alpha-synuclein was found in subpial and periventricular astrocytes in six of the 15 patients with MSA (40%). The structures were confined to the subpial surface of the ventro-lateral part of the spinal cord and brainstem, as well as the subependymal region of the lateral ventricles. They were not visualized by Gallyas-Braak staining, and were immunonegative for ubiquitin and p62. Immunoelectron microscopy revealed that the phosphorylated alpha-synuclein-immunoreactive structures in astrocytes were non-fibrillar and associated with granular and vesicular structures. The extent of phosphorylated alpha-synuclein-immunoreactive astrocytes was correlated with disease duration. No such structures were found in Lewy body disease or controls. Accumulation of phosphorylated alpha-synuclein can occur in subpial and periventricular astrocytes in patients with MSA, especially in those with a long disease duration.

• 出版日期2016-4