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

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• 作者：Jochen Klucken ; Martin Ingelsson ; Youngah Shin ; Michael C. Irizarry ; E. T. Hedley-Whyte ; Matthew P. Frosch ; John H. Growdon ; Pamela J. McLean and Bradley T. Hyman
• 关键词：α ; Synuclein ; Lewy body dementia ; Heat shock proteins
• 刊名：Acta Neuropathologica
• 出版年：2006
• 出版时间：February 2006
• 年：2006
• 卷：111
• 期：2
• 页码：101-108
• 全文大小：533 KB

文摘

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.