The interaction between acquired mitochondrial disease and neurodegeneration

Excerpt

Mitochondrial diseases are traditionally thought of as relatively rare genetic disorders occurring as a result of spontaneous mutation or maternal inheritance with diverse phenotypes but commonly impacting on both muscle and nervous tissue. However, the role of mitochondria is becoming of increasing interest to both clinicians and scientists investigating a wide range of human disease. In particular it is has become apparent that acquired mitochondrial dysfunction may be relevant in a number of neurological disorders and may be particularly relevant in those with a predominant neurodegenerative component. Evolving neurodegenerative pathology is the final common pathway in disorders including Alzheimer’s disease, multiple sclerosis, Parkinson’s and Huntington’s disease, but at present the exact mechanisms that precipitate this process remain obscure. Once established, however, it appears that this process is both irreversible and untreatable, so that understanding its precise mechanism is of considerable importance. Evidence already available suggests that mitochondrial injury may be involved at an early stage, and a clearer understanding of how this may influence later stages of disease may allow important pathological insights, offer a rare opportunity to identify new therapeutic targets, and reduce the profound morbidity and societal impact that neurodegenerative diseases have on both patients and their families. …