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  Current progress with mammalian models of mitochondrial DNA disease

Stewart, J. (2020). Current progress with mammalian models of mitochondrial DNA disease. J Inherit Metab Dis, 44(2), 325-342. doi:10.1002/jimd.12324.

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https://www.ncbi.nlm.nih.gov/pubmed/33099782 (beliebiger Volltext)
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 Urheber:
Stewart, J.1, Autor           
Affiliations:
1Stewart – Mitochondrial Mutations and Genome Co-evolution, Research Groups, Max Planck Institute for Biology of Ageing, Max Planck Society, ou_1942301              

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Schlagwörter: Animals DNA, Mitochondrial/*genetics Disease Models, Animal Humans Mitochondrial Diseases/*genetics/therapy Mutation *animal models *heteroplasmy *homoplasmy *mitochondrial DNA *mitochondrial disease
 Zusammenfassung: Mitochondrial disorders make up a large class of heritable diseases that cause a broad array of different human pathologies. They can affect many different organ systems, or display very specific tissue presentation, and can lead to illness either in childhood or later in life. While the over 1200 genes encoded in the nuclear DNA play an important role in human mitochondrial disease, it has been known for over 30 years that mutations of the mitochondria's own small, multicopy DNA chromosome (mtDNA) can lead to heritable human diseases. Unfortunately, animal mtDNA has resisted transgenic and directed genome editing technologies until quite recently. As such, animal models to aid in our understanding of these diseases, and to explore preclinical therapeutic research have been quite rare. This review will discuss the unusual properties of animal mitochondria that have hindered the generation of animal models. It will also discuss the existing mammalian models of human mtDNA disease, describe the methods employed in their generation, and will discuss recent advances in the targeting of DNA-manipulating enzymes to the mitochondria and how these may be employed to generate new models.

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 Datum: 2020-11-032020-11-03
 Publikationsstatus: Erschienen
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 Identifikatoren: Anderer: 33099782
DOI: 10.1002/jimd.12324
ISSN: 1573-2665 (Electronic)0141-8955 (Linking)
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Titel: J Inherit Metab Dis
Genre der Quelle: Zeitschrift
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Affiliations:
Ort, Verlag, Ausgabe: -
Seiten: - Band / Heft: 44 (2) Artikelnummer: - Start- / Endseite: 325 - 342 Identifikator: -