脂质体
脂类学
脂毒性
脂质代谢
氧化应激
线粒体
4-羟基壬醛
脂肪酸
β氧化
脂肪酸代谢
生物
肉碱
化学
生物化学
脂质过氧化
内分泌学
胰岛素抵抗
胰岛素
作者
Inês M. S. Guerra,Helena Beatriz Ferreira,Tânia Melo,Hugo Rocha,Sónia Moreira,Luísa Diogo,M. Rosário M. Domingues,Ana S. P. Moreira
标识
DOI:10.3390/ijms232213933
摘要
Fatty acid oxidation disorders (FAODs) are inborn errors of metabolism (IEMs) caused by defects in the fatty acid (FA) mitochondrial β-oxidation. The most common FAODs are characterized by the accumulation of medium-chain FAs and long-chain (3-hydroxy) FAs (and their carnitine derivatives), respectively. These deregulations are associated with lipotoxicity which affects several organs and potentially leads to life-threatening complications and comorbidities. Changes in the lipidome have been associated with several diseases, including some IEMs. In FAODs, the alteration of acylcarnitines (CARs) and FA profiles have been reported in patients and animal models, but changes in polar and neutral lipid profile are still scarcely studied. In this review, we present the main findings on FA and CAR profile changes associated with FAOD pathogenesis, their correlation with oxidative damage, and the consequent disturbance of mitochondrial homeostasis. Moreover, alterations in polar and neutral lipid classes and lipid species identified so far and their possible role in FAODs are discussed. We highlight the need of mass-spectrometry-based lipidomic studies to understand (epi)lipidome remodelling in FAODs, thus allowing to elucidate the pathophysiology and the identification of possible biomarkers for disease prognosis and an evaluation of therapeutic efficacy.
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