Abstract
Many neurodegenerative diseases feature mitochondrial dysfunction. This includes both common sporadic and rare Mendelian disorders. In some cases, mitochondrial defects mediate disease pathology, and in others, the defects actually initiate and drive the disease. Understanding the causes and consequences of mitochondrial dysfunction provides insight into neurodegenerative diseases and reveals potential therapeutic targets that could lead to new treatments. This chapter provides an overview of mitochondrial function and its role in cell physiology, presents current perceptions about the relevance of mitochondrial and bioenergetic dysfunction to neurodegenerative diseases, and discusses targeting mitochondria for therapeutic purposes.
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