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This model is especially appealing because it delineates donor mtDNA from the original nuclear background, allowing for the study of mitochondrial mutation-dependent differences in isolation ( Transmitochondrial cybrids are generated by a cytoplasmic fusion of o cells from parent cell lines with enucleated cells from donor cell lines and has allowed for studies on the heteroplasmic threshold, mtDNA-nDNA compatibility (Swerdlow, 2007), mtDNA segregation (Jenuth et al., 1996, 1997), and most importantly for studying various disorders of the mitochondria such as LHON (Iyer, 2013) and LS (Iyer et al., 2009b, 2012)

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