Molecular Pathology of Lewy Body Diseases
Beyer, Katrin (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Domingo-Sàbat, Montse (Institut Germans Trias i Pujol. Hospital Universitari Germans Trias i Pujol)
Ariza Fernández, A (Universitat Autònoma de Barcelona. Departament de Ciències Morfològiques)
Date: |
2009 |
Abstract: |
Lewy body diseases are characterized by the presence of Lewy bodies, alphasynuclein(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 ubiquitinproteasome system and the autophagy-lysosome pathway 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. |
Rights: |
Aquest document està subjecte a una llicència d'ús Creative Commons. Es permet la reproducció total o parcial, la distribució, la comunicació pública de l'obra i la creació d'obres derivades, fins i tot amb finalitats comercials, sempre i quan es reconegui l'autoria de l'obra original. |
Language: |
Anglès |
Document: |
Article ; recerca ; Versió publicada |
Subject: |
Lewy body diseases ;
Parkinson disease ;
Dementia with Lewy bodies ;
Alphasynuclein ;
Molecular chaperones ;
Mitochondrial dysfunction ;
Proteosomal dysfunction ;
Alternative splicing ;
Differential isoform expression |
Published in: |
International journal of molecular sciences, Vol. 10 (2009) , p. 724-745, ISSN 1422-0067 |
DOI: 10.3390/ijms10030724
PMID: 19399218
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Record created 2016-03-04, last modified 2024-05-09