Fatty acid abnormalities in cystic fibrosis–the missing link for a cure?

IF 4.6 2区综合性期刊 Q1 MULTIDISCIPLINARY SCIENCES

iScience Pub Date : 2024-11-15 DOI:10.1016/j.isci.2024.111153

Sławomira Drzymała-Czyż , Jarosław Walkowiak , Carla Colombo , Gianfranco Alicandro , Olav Trond Storrösten , Magnhild Kolsgaard , Egil Bakkeheim , Birgitta Strandvik

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Abstract

The care for cystic fibrosis (CF) has dramatically changed with the development of modulators, correctors, and potentiators of the CFTR molecule, which lead to improved clinical status of most people with CF (pwCF). The modulators influence phospholipids and ceramides, but not linoleic acid (LA) deficiency, associated with more severe phenotypes of CF. The LA deficiency is associated with upregulation of its transfer to arachidonic acid (AA). The AA release from membranes is increased and associated with increase of pro-inflammatory prostanoids and the characteristic inflammation is present before birth and bacterial infections. Docosahexaenoic acid is often decreased, especially in associated liver disease Some endogenously synthesized fatty acids are increased. Cholesterol and ceramide metabolisms are disturbed. The lipid abnormalities are present at birth, and before feeding in transgenic pigs and ferrets. This review focus on the lipid abnormalities and their associations to clinical symptoms in CF, based on clinical studies and experimental research.

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囊性纤维化的脂肪酸异常--治愈的缺失环节？

随着 CFTR 分子的调节剂、矫正剂和增效剂的开发，囊性纤维化（CF）的治疗发生了巨大变化，大多数 CF 患者（pwCF）的临床状况得到了改善。调节剂会影响磷脂和神经酰胺，但不会影响亚油酸（LA）缺乏，亚油酸缺乏与 CF 的严重表型有关。亚油酸缺乏与亚油酸向花生四烯酸（AA）转移的上调有关。从膜中释放的 AA 会增加，并与促炎前列腺素的增加有关，在出生和细菌感染前就会出现特征性炎症。二十二碳六烯酸通常会减少，尤其是在伴有肝病的情况下。胆固醇和神经酰胺代谢紊乱。转基因猪和雪貂会在出生时和喂养前出现脂质异常。本综述以临床研究和实验研究为基础，重点讨论脂质异常及其与 CF 临床症状的关系。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

iScience Multidisciplinary-Multidisciplinary

CiteScore

7.20

自引率

1.70%

发文量

1972

审稿时长

6 weeks

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