Acta Neuropathologica

, Volume 99, Issue 4, pp 352–357

α-Synuclein immunoreactivity in dementia with Lewy bodies: morphological staging and comparison with ubiquitin immunostaining

  • E. Gómez-Tortosa
  • K. Newell
  • M. C. Irizarry
  • J. L. Sanders
  • B. T. Hyman
Regular paper

DOI: 10.1007/s004010051135

Cite this article as:
Gómez-Tortosa, E., Newell, K., Irizarry, M. et al. Acta Neuropathol (2000) 99: 352. doi:10.1007/s004010051135

Abstract

α-Synuclein is a presynaptic protein recently identified as a specific component of Lewy bodies (LB) and Lewy neurites. The aim of this study was to assess the morphology and distribution of α-synuclein immunoreactivity in cases of dementia with LB (DLB), and to compare α-synuclein with ubiquitin immunostaining. We examined substantia nigra, paralimbic regions (entorhinal cortex, cingulate gyrus, insula and hippocampus), and neocortex (frontal and occipital association cortices) with double α-synuclein and ubiquitin immunostaining in 25 cases meeting neuropathological criteria for DLB. α-Synuclein immunostaining was more specific than ubiquitin immunostaining in that it differentiated LB from globose tangles. It was also slightly more sensitive, staining 4–5% more intracytoplasmic structures, especially diffuse α-synuclein deposits that were ubiquitin negative. In addition to LB, α-synuclein staining showed filiform and globose neurites in the substantia nigra, CA2–3 regions of the hippocampus, and entorhinal cortex. A spectrum of α-synuclein staining was seen in substantia nigra: from diffuse “cloud-like” inclusions to aggregated intracytoplasmic inclusions with variable ubiquitin staining to classic LB. We hypothesize that these represent different stages in LB formation.

Key words Lewy bodies α-Synuclein Ubiquitin Dementia Parkinson’s disease 

Copyright information

© Springer-Verlag Berlin Heidelberg 2000

Authors and Affiliations

  • E. Gómez-Tortosa
    • 1
  • K. Newell
    • 1
  • M. C. Irizarry
    • 1
  • J. L. Sanders
    • 1
  • B. T. Hyman
    • 1
  1. 1.Neurology Department, Massachusetts General Hospital, Charlestown, Massachusetts, USAUS
  2. 2.Alzheimer’s Research Unit, Massachusetts General Hospital East 149 13th St., Room 6405, Charlestown, MA 02129, USA e-mail: b_hyman@helix.mgh.harvard.edu, Tel.: +1-617-7262299, Fax: +1-617-7265677US

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