“deletor” mice do not, however, show premature aging, indicating that subtle accumulation of mtDNA deletions and progressive respiratory chain dysfunction are not sufficient to accelerate aging
muscles of the mice faithfully replicate all of the key histological, genetic, and biochemical features of PEO patients
mice have progressive deficiency of cytochrome c oxidase in distinct neuronal populations
generated transgenic mice expressing mouse Twinkle with PEO patient mutations
Multiple mtDNA deletions accumulate in the tissues of these mice, resulting in progressive respiratory dysfunction
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