Neurodegenerative Disorders: Spotlight on Sphingolipids.
Neurodegenerative Disorders: Spotlight on Sphingolipids.
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DOI：
10.3390/ijms222111998
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发表时间：
2021-11-05
影响因子：
5.6
通讯作者：
Vos M
中科院分区：
文献类型：
--
作者：
Mandik F;Vos M
神经退行性疾病是神经系统的不治之症，导致脑区和神经元亚型的进行性丧失，这与可能与受影响脑区有关的症状增加有关。在许多神经退行性疾病中出现的关键发现是蛋白质沉积和线粒体损伤，这主要影响能量产生和线粒体自噬。已经在各种神经退行性疾病中鉴定出几种致病基因突变;然而，很大一部分被认为是散发的。在过去的十年中，将脂质，特别是鞘脂与神经退行性疾病联系起来的研究已经显示了这些鞘脂在潜在发病机制中的重要性。鞘脂是由连接至脂肪酸的鞘氨醇碱和亲水性头部基团组成的生物活性脂质。它们参与各种细胞过程，如细胞生长，凋亡和自噬，并且是大脑的重要组成部分。在这篇综述中，我们将涵盖证明鞘脂与神经退行性疾病相关性的关键发现，并将重点关注神经退行性疾病与脑铁积累和帕金森病。
Neurodegenerative diseases are incurable diseases of the nervous system that lead to a progressive loss of brain areas and neuronal subtypes, which is associated with an increase in symptoms that can be linked to the affected brain areas. The key findings that appear in many neurodegenerative diseases are deposits of proteins and the damage of mitochondria, which mainly affect energy production and mitophagy. Several causative gene mutations have been identified in various neurodegenerative diseases; however, a large proportion are considered sporadic. In the last decade, studies linking lipids, and in particular sphingolipids, to neurodegenerative diseases have shown the importance of these sphingolipids in the underlying pathogenesis. Sphingolipids are bioactive lipids consisting of a sphingoid base linked to a fatty acid and a hydrophilic head group. They are involved in various cellular processes, such as cell growth, apoptosis, and autophagy, and are an essential component of the brain. In this review, we will cover key findings that demonstrate the relevance of sphingolipids in neurodegenerative diseases and will focus on neurodegeneration with brain iron accumulation and Parkinson’s disease.
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