Abstract
Dysfunction of the mitochondrial respiratory chain has been associated with a wide range of human diseases ranging from diabetes to cardiomyopathy. Mutations in a number of nuclear as well as mitochondrial genes have been implicated in causing these diseases. Several animal models have now been created which reproduce some of the clinical pathology observed in human patients suffering from OXPHOS disorders. In this chapter we review some of these animal models of OXPHOS disorders and how they have led to a further understanding of both mitochondrial respiratory chain function and dysfunction.
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Hance, N., Larsson, NG. (2004). Animal Models of OXPHOS Disorders. In: Oxidative Phosphorylation in Health and Disease. Medical Intelligence Unit. Springer, Boston, MA. https://doi.org/10.1007/0-387-26992-4_9
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DOI: https://doi.org/10.1007/0-387-26992-4_9
Publisher Name: Springer, Boston, MA
Print ISBN: 978-0-306-48232-8
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