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The NDUFS4 Knockout Mouse: A Dual Threat Model of Childhood Mitochondrial Disease and Normative Aging. | LitMetric

AI Article Synopsis

  • * Research on these mice has improved understanding of mitochondrial dysfunction and led to potential treatments to alleviate disease symptoms.
  • * The chapter covers practical methods for managing these mice, including breeding, genotyping, and weaning, which can be complex due to their serious health issues.

Article Abstract

Mice missing the Complex I subunit NADH:Ubiquinone Oxidoreductase Fe-S Protein 4 (NDUFS4) of the electron transport chain are a leading model of the severe mitochondrial disease Leigh syndrome. These mice have enabled a better understanding of mitochondrial dysfunction in human disease, as well as in the discovery of interventions that can potentially suppress mitochondrial disease manifestations. In addition, increasing evidence suggests significant overlap between interventions that increase survival in NDUFS4 knockout mice and that extend life span during normative aging. This chapter discusses the practical aspects of handling and studying these mice, which can be challenging due to their severe disease phenotype. Common procedures such as breeding, genotyping, weaning, or treating these transgenic mice are also discussed.

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Source
http://dx.doi.org/10.1007/978-1-0716-1270-5_10DOI Listing

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