Publisher Summary This chapter investigates mitochondrial respiratory complex activity in
mitochondria isolated from human cells and tissues. Mitochondrial cytopathies are a
heterogeneous group of multisystem disorders predominantly affecting skeletal and cardiac
muscle and the central nervous system, but are associated with a broad spectrum of other
clinical phenotypes, including neurodegenerative disease. In many patients, the impairment
of mitochondrial respiratory chain function is due to a mutation in mitochondrial DNA …