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Acta Neuropathologica

, Volume 104, Issue 1, pp 7–11 | Cite as

Concurrence of α-synuclein and tau brain pathology in the Contursi kindred

  • John E. Duda
  • Benoit I. Giasson
  • Meghann E. Mabon
  • Douglas C. Miller
  • Lawrence I. Golbe
  • Virginia M.-Y. Lee
  • John Q. Trojanowski
Express Communication

Abstract.

Previous genetic analysis of the familial Parkinson's disease Contursi kindred led to the identification of an Ala53Thr pathogenic mutation in the α-synuclein gene. We have re-examined one of the original brains from this kindred using new immunohistochemical reagents, thioflavin S staining and immunoelectron microscopy. Surprisingly, we uncovered a dense burden of α-synuclein neuritic pathology and rare Lewy bodies. Immunoelectron microscopy demonstrated fibrillar α-synuclein-immunoreactive aggregates. Unexpected tau neuritic and less frequent perikaryal inclusions were also observed. Some inclusions were comprised of both proteins with almost complete spatial disparity. We suggest that it is important to recognize that the neurodegenerative process caused by the Ala53Thr mutation in α-synuclein is not identical to that seen in typical idiopathic Parkinson's disease brains.

Neuropathology α-Synuclein Parkinson's disease Histopathology Lewy bodies 

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Copyright information

© Springer-Verlag 2002

Authors and Affiliations

  • John E. Duda
    • 1
  • Benoit I. Giasson
    • 1
  • Meghann E. Mabon
    • 1
  • Douglas C. Miller
    • 2
  • Lawrence I. Golbe
    • 3
  • Virginia M.-Y. Lee
    • 1
  • John Q. Trojanowski
    • 1
  1. 1.Center for Neurodegenerative Disease Research, Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, 3rd Floor Maloney Building, Philadelphia, PA 19104USA
  2. 2.Department of Pathology, Division of Neuropathology, NYU School of Medicine, New York, New YorkUSA
  3. 3.Department of Neurology, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, New JerseyUSA

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