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Related Experiment Video

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Broad AOX expression in a genetically tractable mouse model does not disturb normal physiology.

Marten Szibor1,2,3, Praveen K Dhandapani1,2, Eric Dufour2

  • 1Institute of Biotechnology, FI-00014 University of Helsinki, Finland.

Disease Models & Mechanisms
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PubMed
Summary

Mice engineered to express alternative oxidases (AOX) showed robust resistance to mitochondrial respiratory chain inhibitors and cyanide poisoning. This AOX mouse model offers a new tool for studying mitochondrial diseases.

Keywords:
Alternative oxidaseMitochondriaMitochondrial diseaseRespiratory chain

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Area of Science:

  • Mitochondrial biology
  • Biochemistry
  • Genetics

Background:

  • Alternative oxidases (AOX) are present in plants and lower organisms but not mammals, playing a role in mitochondrial respiration.
  • AOX bypasses the classical respiratory chain, limiting reactive oxygen species production and maintaining homeostasis.
  • Previous studies utilized AOX in cell and animal models to explore mitochondrial impairments.

Purpose of the Study:

  • To create a genetically tractable mouse model for ubiquitous alternative oxidase (AOX) expression.
  • To investigate the physiological effects and protective capabilities of AOX in vivo.
  • To establish a tool for studying mitochondrial disease aetiology.

Main Methods:

  • Targeted insertion of Ciona intestinalis AOX into the Rosa26 locus for ubiquitous expression in mice.
  • Phenotypic analysis including respiratory complex assessment, oxygen consumption, and metabolomics.
  • Evaluation of AOX-mediated resistance to respiratory chain inhibitors and cyanide toxicity.

Main Results:

  • The AOX-Rosa26 mouse model displayed subtle physiological effects and normal overall physiology.
  • Mitochondria exhibited enhanced resistance to respiratory chain inhibitors.
  • Mice demonstrated significant protection against lethal doses of cyanide.

Conclusions:

  • The AOX-Rosa26 mouse is a viable model for ubiquitous AOX expression with minimal physiological impact.
  • This model provides robust protection against mitochondrial respiratory chain dysfunction.
  • The AOX-Rosa26 mouse serves as a valuable tool for in vivo research into mitochondrial diseases and respiratory control.