Acta Neuropathologica

, Volume 95, Issue 2, pp 117–122 | Cite as

Amyloid plaques in Guam amyotrophic lateral sclerosis/ parkinsonism-dementia complex contain species of Aβ similar to those found in the amyloid plaques of Alzheimer’s disease and pathological aging

  • M. L. Schmidt
  • V. M.-Y. Lee
  • T. Saido
  • D. Perl
  • T. Schuck
  • T. Iwatsubo
  • J. Q. Trojanowski
Express communication

Abstract

The Guamanian amyotrophic lateral sclerosis/parkinsonism-dementia complex (ALS/PDC) is characterized by abundant neurofibrillary pathology and neuron loss. In contrast to Alzheimer’s disease (AD), where extensive neurofibrillary lesions always occur with deposits of Aβ in numerous amyloid plaques, Aβ-rich amyloid plaques are absent or rare in most ALS/PDC patients. To characterize the amyloid plaques in the latter patients, we probed plaque-rich sections of their brains by immunohistochemistry using well-characterized antibodies to specific epitopes in the N and C termini of Aβ as well as to defined epitopes in hyperphosphorylated tau (PHFtau). The results indicate that the species of Aβ in the amyloid plaques of ALS/PDC patients resemble those detected in the amyloid plaques of cognitively intact subjects with pathological aging as well as patients with AD. However, the paucity of PHFtau-positive neurites in the ALS/PDC plaques suggests that they reflect pathological aging rather than AD.

Key words Amyloid plaques Amyotrophic lateral sclerosis/parkinsonism-dementia complex Guam End-terminus-specific anti-amyloid β protein antibodies N-terminal modification of amyloid β protein 

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

Copyright information

© Springer-Verlag Berlin Heidelberg 1998

Authors and Affiliations

  • M. L. Schmidt
    • 1
  • V. M.-Y. Lee
    • 1
  • T. Saido
    • 2
  • D. Perl
    • 3
  • T. Schuck
    • 1
  • T. Iwatsubo
    • 4
  • J. Q. Trojanowski
    • 1
  1. 1.Center for Neurodegenerative Disease Research, University of Pennsylvania, 3rd Floor Maloney, 3600 Spruce Street, Philadelphia, PA 19104-4283, USA Fax: 1-215-6349-5909US
  2. 2.Tokyo Metropolitan Geriatric Institute, Tokyo, JapanJP
  3. 3.Mt. Sinai Medical School, New York, USAUS
  4. 4.Tokyo University, Tokyo, JapanJP

Personalised recommendations