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Mitochondrial dysfunction in cystic fibrosis Cystic Fibrosis (CF) is a lethal autosomal recessive disease where mutation F508 (loss of phenylalanine residue at position 508) in the gene encoding Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) is responsible for the abnormal mucus secretions (Rowe et al., 2005)

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