Acta Neuropathologica

, Volume 111, Issue 2, pp 101–108

Clinical and biochemical correlates of insoluble α-synuclein in dementia with Lewy bodies

  • Jochen Klucken
  • Martin Ingelsson
  • Youngah Shin
  • Michael C. Irizarry
  • E. T. Hedley-Whyte
  • Matthew P. Frosch
  • John H. Growdon
  • Pamela J. McLean
  • Bradley T. Hyman
Original Paper

DOI: 10.1007/s00401-005-0027-7

Cite this article as:
Klucken, J., Ingelsson, M., Shin, Y. et al. Acta Neuropathol (2006) 111: 101. doi:10.1007/s00401-005-0027-7

Abstract

α-Synuclein is a major constituent of Lewy bodies, the fibrillar aggregates that form within neurons in Parkinson’s disease and dementia with Lewy bodies (DLB). Recent biochemical data show that α-synuclein accumulates in Parkinson’s disease in a detergent insoluble form. We now examine the relationship between detergent insoluble α-synuclein and the presence of Lewy bodies, clinical measures of dementia and biochemical parameters in a series of individuals with DLB. We found that Triton X-100 insoluble α-synuclein enriched nearly twofold in the temporal cortex of patients with DLB compared to age-matched controls. By contrast the total amount of α-synuclein protein was unchanged. Surprisingly, the degree of Triton X-100 insoluble α-synuclein did not correlate with either the duration of illness or the number of Lewy bodies counted using stereological methods from an adjacent block of tissue. However, the Triton X-100 soluble fraction of α-synuclein did correlate strongly with the expression of several heat shock proteins (HSPs) in DLB but not control cases, suggesting a coordinated HSP response in DLB neocortex.

Keywords

α-Synuclein Lewy body dementia Heat shock proteins 

Copyright information

© Springer-Verlag 2006

Authors and Affiliations

  • Jochen Klucken
    • 1
    • 3
  • Martin Ingelsson
    • 1
  • Youngah Shin
    • 1
  • Michael C. Irizarry
    • 1
  • E. T. Hedley-Whyte
    • 2
  • Matthew P. Frosch
    • 2
  • John H. Growdon
    • 1
  • Pamela J. McLean
    • 1
  • Bradley T. Hyman
    • 1
  1. 1.Department of NeurologyMassGeneral Institute for Neurodegenerative disease, Massachusetts General HospitalCharlestownUSA
  2. 2.Department of NeuropathologyMassGeneral Institute for Neurodegenerative disease, Massachusetts General HospitalCharlestownUSA
  3. 3.Clinic and Policlinic for NeurologyUniversity of RegensburgRegensburgGermany

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