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Accumulation of NACP/α-synuclein in Lewy body disease and multiple system atrophy
  1. Mikio Shojia,
  2. Yasuo Harigayaa,
  3. Atsushi Sasakib,
  4. Kenji Uédac,
  5. Koji Ishigurod,
  6. Etsuro Matsubaraa,
  7. Mitsunori Watanabea,
  8. Masaki Ikedaa,
  9. Mitsuyasu Kanaia,
  10. Yasushi Tomidokoroa,
  11. Masami Shizukaa,
  12. Masakuni Amarie,
  13. Kenji Kosakaf,
  14. Yoichi Nakazatob,
  15. Koichi Okamotoa,
  16. Shunsaku Hiraig
  1. aDepartment of Neurology, Gunma University School of Medicine, Japan, bFirst Department of Pathology, Gunma University School of Medicine, Japan, cDepartment of Neurochemistry, Tokyo Institute of Psychiatry, Japan, dDepartment of Neurology, Maebashi Red Cross Hospital, Japan, eGeriatrics Research Institute and Hospital, Japan, fDepartment of Psychiatry, Yokohama City University, School of Medicine, Japan, gTokyo Metropolitan Neurological Hospital, Japan
  1. Dr Mikio Shoji, Department of Neurology, Gunma University School of Medicine, 3–39–22 Showamachi, Maebashi, Gunma 371–8511, Japan email mshoji{at}akagi.sb.gunma-u.ac.jp

Abstract

OBJECTIVES NACP/α-synuclein is an aetiological gene product in familial Parkinson's disease. To clarify the pathological role of NACP/α-synuclein in sporadic Parkinson's disease and other related disorders including diffuse Lewy body disease (DLBD) and multiple system atrophy (MSA), paraffin sections were examined immunocytochemically using anti-NACP/α-synuclein antibodies.

METHODS A total of 58 necropsied brains, from seven patients with Parkinson's disease, five with DLBD, six with MSA, 12 with Alzheimer's disease, one with Down's syndrome, one with amyotrophic lateral sclerosis (ALS), three with ALS and dementia, one with Huntington's disease, two with progressive supranuclear palsy (PSP), one with Pick's disease, one with myotonic dystrophy, and three with late cerebellar cortical atrophy (LCCA), and 15 elderly normal controls were examined.

RESULTS In addition to immunoreactive Lewy bodies, widespread accumulation of NACP/α-synuclein was found in neurons and astrocytes from the brainstem and basal ganglia to the cerebral cortices in Parkinson's disease/DLBD. NACP/α-synuclein accumulates in oligodendrocytes from the spinal cord, the brain stem to the cerebellar white matter, and inferior olivary neurons in MSA. These widespread accumulations were not seen in other types of dementia or spinocerebellar ataxia.

CONCLUSION Completely different types of NACP/α-synuclein accumulation in Parkinson's disease/DLBD and MSA suggest that accumulation is a major step in the pathological cascade of both diseases and provides novel strategies for the development of therapies.

  • Parkinson's disease
  • multiple system atrophy
  • NACP
  • α-synuclein

https://doi.org/10.1136/jnnp.68.5.605

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