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Int. J. Mol. Sci. 2009, 10(3), 724-745; doi:10.3390/ijms10030724
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Molecular Pathology of Lewy Body Diseases

* ,
 and
Received: 18 December 2008 / Revised: 3 February 2009 / Accepted: 23 February 2009 / Published: 26 February 2009
(This article belongs to the Special Issue Advances in Molecular Neuropathology)
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Abstract

Lewy body diseases are characterized by the presence of Lewy bodies, alpha-synuclein(AS)-positive inclusions in the brain. Since their main component is conformationally modified AS, aggregation of the latter is thought to be a key pathogenic event in these diseases. The analysis of inclusion body constituents gives additional information about pathways also involved in the pathology of synucleinopathies. Widespread mitochondrial dysfunction is very closely related to disease development. The impairment of protein degradation pathways, including both the ubiquitin-proteasome system and the autophagy-lysosomepathway also play an important role during the development of Lewy body diseases. Finally, differential expression changes of isoforms corresponding to genes primarily involved in Lewy body formation point to alternative splicing as another important mechanism in the development of Parkinson’s disease, as well as dementia with Lewy bodies. The present paper attempts to give an overview of recent molecular findings related to the pathogenesis of Lewy body diseases.
Keywords: Lewy body diseases; Parkinson disease; dementia with Lewy bodies; alpha-synuclein; molecular chaperones; mitochondrial dysfunction; proteosomal dysfunction; alternative splicing; differential isoform expression Lewy body diseases; Parkinson disease; dementia with Lewy bodies; alpha-synuclein; molecular chaperones; mitochondrial dysfunction; proteosomal dysfunction; alternative splicing; differential isoform expression
This is an open access article distributed under the Creative Commons Attribution License which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

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Beyer, K.; Domingo-Sàbat, M.; Ariza, A. Molecular Pathology of Lewy Body Diseases. Int. J. Mol. Sci. 2009, 10, 724-745.

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