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doi: 10.1093/alcalc/agr038.agr038 [DOI] [PubMed] [Google Scholar] 64.Lieber C

1.3 Evolution and development of preclinical models of mitochondrial-driven diseases The development of preclinical models for mitochondrial-driven diseases has undergone significant evolution, marked by key milestones (Figure 2) that have shaped the field, such as: i) the development of technologies to study mtDNA mutations in a controlled nuclear background, enabling the functional analysis of the consequences of mitochondrial defects, ii) the successful integration of mitochondria from one species into another, allowing the study of the mitochondrial abnormalities to disease progression, iii) the creation of models where mtDNA from one strain is introduced into the nuclear background of another through breeding, helping dissect mtDNA-nDNA interactions and consequences of mismatch, and lastly (iv) the development of a model that reciprocally exchanges nuclei between embryos with different mtDNA backgrounds, revealing the independent effects of mtDNA while controlling the nDNA background