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However, the common pathogenic mechanisms of neuronal death are still largely unknown. Recently, mitochondrial dysfunction has emerged as a potential \u2018lowest common denominator\u2019 linking these disorders. In this review, we discuss the body of evidence supporting the role of mitochondria in the pathogenesis of hereditary neurodegenerative diseases. We summarize the principal features of genetic diseases caused by abnormalities of mitochondrial proteins encoded by the mitochondrial or the nuclear genomes. We then address genetic diseases where mutant proteins are localized in multiple cell compartments, including mitochondria and where mitochondrial defects are likely to be directly caused by the mutant proteins. Finally, we describe examples of neurodegenerative disorders where mitochondrial dysfunction may be \u2018secondary\u2019 and probably concomitant with degenerative events in other cell organelles, but may still play an important role in the neuronal decay. Understanding the contribution of mitochondrial dysfunction to neurodegeneration and its pathophysiological basis will significantly impact our ability to develop more effective therapies for neurodegenerative diseases.<\/jats:p>","DOI":"10.1111\/j.1471-4159.2006.03990.x","type":"journal-article","created":{"date-parts":[[2006,6,19]],"date-time":"2006-06-19T07:43:05Z","timestamp":1150702985000},"page":"1659-1675","source":"Crossref","is-referenced-by-count":148,"title":["The role of mitochondria in inherited neurodegenerative diseases"],"prefix":"10.1111","volume":"97","author":[{"given":"Jennifer Q.","family":"Kwong","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M. 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