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366 The primary mitochondrial diseases caused by nDNA mutations Given that the majority of mitochondrial proteins are encoded by nDNA, mitochondrial diseases resulting from nDNA mutations encompass not only disturbances in OXPHOS but also defects in various structural or functional proteins essential for mtDNA maintenance, mitochondrial function and structure, and mito-nuclear communication

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Antioxidant imbalance in the lungs of cystic fibrosis transmembrane conductance regulator protein mutant mice. American Journal of Physiology-Lung Cellular and Molecular Physiology 281.1 (2001): L31-L38