From Pluripotency to Differentiation: The Role of mtDNA in Stem Cell Models of Mitochondrial Diseases

Joao M Facucho-Oliveira, Tejal Kulkarni, Gisela Machado-Oliveira, Justin Charles St John

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

    Abstract

    Embryonic stem cells (ESCs) are characterized by pluripotency, selfrenewal and unlimited proliferation representing a limitless supply of cells for therapy. Moreover, ESCs represent a unique experimental model to investigate the basic principles of mammalian cell differentiation. ESCs are very useful for in-depth analysis of the development of the mitochondrial complement as the cells activate aerobic metabolism during differentiation. Induced pluripotent stem cells (iPSCs), which are reprogrammed somatic cells, appear to have identical properties to those of ESCs. They will certainly be a fundamental tool to establish human models for specific diseases. Nevertheless, the generation of iPSCs through reprogramming of mouse and human differentiated adult cells containing a mature mitochondrial complement requires a complete reprogramming of the cytoplasm to acquire the ''pluripotent'' mitochondrial network typical of undifferentiated ESCs.

    Original languageEnglish
    Title of host publicationMitochondrial DNA, Mitochondria, Disease and Stem Cells
    EditorsJustin Charles St John
    Place of PublicationUnited States
    PublisherHumana Press
    Pages87-118
    Number of pages32
    ISBN (Electronic)9781627031011
    ISBN (Print)9781627031004
    DOIs
    Publication statusPublished - 1 Jan 2013

    Cite this

    Facucho-Oliveira, J. M., Kulkarni, T., Machado-Oliveira, G., & St John, J. C. (2013). From Pluripotency to Differentiation: The Role of mtDNA in Stem Cell Models of Mitochondrial Diseases. In J. C. St John (Ed.), Mitochondrial DNA, Mitochondria, Disease and Stem Cells (pp. 87-118). Humana Press. https://doi.org/10.1007/978-1-62703-101-1_5, https://doi.org/10.1007/978-1-62703-101-1_5