Role of Mitochondria in Parkinson Disease
The cause of the selective degeneration of nigrostriatal neurons in Parkinson disease (PD) has remained largely unknown. Exceptions include rare missense mutations in the alpha-synuclein gene on chromosome 4, a potentially pathogenic mutation affecting the ubiquitin pathway, and mutations in the par...
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Published in | Biological chemistry Vol. 380; no. 7-8; pp. 865 - 870 |
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Main Authors | , , , , |
Format | Journal Article |
Language | English |
Published |
Germany
Walter de Gruyter
01.07.1999
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Subjects | |
Online Access | Get full text |
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Summary: | The cause of the selective degeneration of nigrostriatal neurons in Parkinson disease (PD) has remained largely unknown. Exceptions include rare missense mutations in the alpha-synuclein gene on chromosome 4, a potentially pathogenic mutation affecting the ubiquitin pathway, and mutations in the parkin gene on chromosome 6. However, unlike classical PD, the latter syndrome is not associated with the formation of typical Lewy bodies. In contrast, a biochemical defect of complex I of the mitochondrial respiratory chain has been described in a relatively large group of confirmed PD cases. Recent cybrid studies indicate that the complex I defect in PD has a genetic cause and that it may arise from mutations in the mitochondrial DNA. Sequence analysis of the mitochondrial genome supports the view that mitochondrial point mutations are involved in PD pathogenesis. However, although mitochondria function as regulators in several known forms of cell death, their exact involvement in PD has remained unresolved. This is of relevance because classical apoptosis does not appear to play a major role in the degeneration of the parkinsonian nigra. |
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Bibliography: | ark:/67375/QT4-GCVPD4Z8-N istex:01E05AF8941EB95AD56AA6D19B332E5AF63440A7 bc.1999.106.pdf ArticleID:bchm.380.7-8.865 ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-1 content type line 23 ObjectType-Feature-3 ObjectType-Review-1 |
ISSN: | 1431-6730 1437-4315 |
DOI: | 10.1515/BC.1999.106 |