Cystic fibrosis (CF) is the most common lethal inherited disorder among Caucasians and results from mutation in the gene encoding the CF transmembrane conductance regulator. In addition to its multisystem clinical effects, the disease is characterized by increased proinflammatory mediators and oxidant stress, and systemic redox imbalance with reduced glutathione (GSH), together with alterations in circulating and tissue (n-6) and (n-3) fatty acids, particularly a decrease in docosahexaenoic acid. The metabolism of phospholipids and fatty acids is closely related to GSH through the methionine-homocysteine cycle, in which choline via betaine provides methyl groups to regenerate S-adenosylmethionine, important in generating phosphatidylcholine and amino acid precursors for GSH. Current research focuses both on fatty acid supplementations to normalize altered (n-6) to (n-3) fatty acid balance and decrease generation of (n-6) fatty acid-derived inflammatory mediators, and strategies to improve oxidant defenses and redox balance. However, further research is needed before such strategies can be included in clinical care of individuals with CF.

中文翻译：

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囊性纤维化和营养：将磷脂和必需脂肪酸与硫醇代谢联系在一起。

囊性纤维化（CF）是高加索人中最常见的致死性遗传疾病，由CF跨膜电导调节剂编码基因的突变导致。除具有多系统临床作用外，该疾病还具有促炎性介质和氧化应激增加，系统性氧化还原失衡，谷胱甘肽（GSH）减少以及循环和组织（n-6）和（n-3）脂肪改变的特征。酸，特别是二十二碳六烯酸的减少。磷脂和脂肪酸的代谢通过蛋氨酸-高半胱氨酸循环与GSH密切相关，其中通过甜菜碱的胆碱提供了甲基来再生S-腺苷甲硫氨酸，这对于生成GSH的磷脂酰胆碱和氨基酸前体很重要。当前的研究集中在补充脂肪酸以使改变的（n-6）到（n-3）脂肪酸平衡正常化并减少（n-6）脂肪酸衍生的炎症介质的产生，以及改善氧化剂防御和氧化还原平衡的策略。 。但是，在将此类策略纳入CF患者的临床护理之前，需要进一步的研究。