Acta Neuropathologica

, Volume 107, Issue 4, pp 292–298

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

  • Makoto Nishie
  • Fumiaki Mori
  • Hideo Fujiwara
  • Masato Hasegawa
  • Makoto Yoshimoto
  • Takeshi Iwatsubo
  • Hitoshi Takahashi
  • Koichi Wakabayashi
Regular Paper

DOI: 10.1007/s00401-003-0811-1

Cite this article as:
Nishie, M., Mori, F., Fujiwara, H. et al. Acta Neuropathol (2004) 107: 292. doi:10.1007/s00401-003-0811-1

Abstract

We immunohistochemically examined the brain and peripheral sympathetic ganglia from eight patients with multiple system atrophy (MSA), using an antibody specific for phosphorylated α-synuclein (anti-PSer129). Phosphorylated α-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 α-synuclein in both glial and neuronal cells is a pathological feature in patients suffering from MSA.

Keywords

Multiple system atrophy α-Synucleinopathy Neuronal cytoplasmic inclusions Neuropil threads Phosphorylation 

Copyright information

© Springer-Verlag 2004

Authors and Affiliations

  • Makoto Nishie
    • 1
  • Fumiaki Mori
    • 1
  • Hideo Fujiwara
    • 2
  • Masato Hasegawa
    • 3
  • Makoto Yoshimoto
    • 4
  • Takeshi Iwatsubo
    • 2
  • Hitoshi Takahashi
    • 5
  • Koichi Wakabayashi
    • 1
  1. 1.Department of Neuropathology, Institute of Brain ScienceHirosaki University School of MedicineHirosakiJapan
  2. 2.Department of Neuropathology and Neuroscience, Graduate School of Pharmaceutical ScienceThe University of TokyoTokyoJapan
  3. 3.Department of Molecular Neurobiology, Tokyo Institute of PsychiatryTokyo Metropolitan Organization for Medical ResearchTokyoJapan
  4. 4.Molecular Biology Laboratory, Medicinal Research LaboratoriesTaisho PharmaceuticalsSaitamaJapan
  5. 5.Department of Pathology, Brain Research InstituteNiigata UniversityNiigataJapan

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