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Oxidative Phosphorylation in Health and Disease pp 149–169Cite as

Animal Models of OXPHOS Disorders

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Part of the book series: Medical Intelligence Unit ((MIUN))

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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Authors and Affiliations

  1. Departments of Medical Nutrition and Biosciences Karolinska Institute, Huddinge University Hospital, Huddinge, Sweden

    Nicole Hance & Nils-Göran Larsson

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  1. Nicole Hance
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  2. Nils-Göran Larsson
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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

  • Online ISBN: 978-0-387-26992-4

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