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214 This process is performed in repeated cycles, each of which removes a two-carbon acetyl-coA from the acyl-CoA via four enzymes, the enoyl-CoA hydratase, ketoacyl-CoA thiolase, acyl-CoA dehydrogenase (ACADs) and hydroxyacyl-CoA dehydrogenase (HADH)

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CF patients characteristically exhibit recurrent infections/inflammations of the respiratory tract, which is consistent with an increase in oxidative stress