Accumulation of phosphorylated [alpha]-synuclein in the brain and peripheral ganglia of patients with multiple system atrophy.
Nishie, Makoto 1; Mori, Fumiaki 1; Fujiwara, Hideo 2; Hasegawa, Masato 3; Yoshimoto, Makoto 4; Iwatsubo, Takeshi 2; Takahashi, Hitoshi 5; Wakabayashi, Koichi 1
[Article] Acta Neuropathologica. 107(4):292-298, April 2004.

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: 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.

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