Generation of xenomitochondrial embryonic stem cells for the production of live xenomitochondrial mice

Ian A. Trounce, Jessica Ackerley, Matthew McKenzie

Research output: Chapter in Book/Report/Conference proceedingChapter (Book)Otherpeer-review


The unique features of the mitochondrial genome, such as its high copy number and lack of defined mechanisms of recombination, have hampered efforts to manipulate its sequence to create specific mutations in mouse mtDNA. As such, the generation of in vivo mouse models of mtDNA disease has proved technically challenging. This chapter describes a unique approach to create mitochondrial oxidative phosphorylation (OXPHOS) defects in mouse ES cells by transferring mtDNA from different murid species into Mus musculus domesticus ES cells using cytoplasmic hybrid (“cybrid”) fusion. The resulting “xenocybrid” ES cells carry OXPHOS defects of varying severity, and can be utilized to generate live mouse models of mtDNA disease.

Original languageEnglish
Title of host publicationMitochondrial DNA
Subtitle of host publicationMethods and Protocols
EditorsMatthew McKenzie
Place of PublicationNew York NY USA
PublisherHumana Press
Number of pages11
ISBN (Electronic)9781493930401
ISBN (Print)9781493930395
Publication statusPublished - 2016
Externally publishedYes

Publication series

NameMethods in Molecular Biology
ISSN (Print)1064-3745
ISSN (Electronic)1940-6029


  • Embryonic stem cells
  • Fibroblasts
  • Fusion
  • Mouse
  • Xenocybrid

Cite this