Centre for Neuroscience Studies at Queen's University, Kingston, ON K7L 3N6, Canada.
Int J Mol Sci. 2021 May 28;22(11):5793. doi: 10.3390/ijms22115793.
Sphingolipids are a specialized group of lipids essential to the composition of the plasma membrane of many cell types; however, they are primarily localized within the nervous system. The amphipathic properties of sphingolipids enable their participation in a variety of intricate metabolic pathways. Sphingoid bases are the building blocks for all sphingolipid derivatives, comprising a complex class of lipids. The biosynthesis and catabolism of these lipids play an integral role in small- and large-scale body functions, including participation in membrane domains and signalling; cell proliferation, death, migration, and invasiveness; inflammation; and central nervous system development. Recently, sphingolipids have become the focus of several fields of research in the medical and biological sciences, as these bioactive lipids have been identified as potent signalling and messenger molecules. Sphingolipids are now being exploited as therapeutic targets for several pathologies. Here we present a comprehensive review of the structure and metabolism of sphingolipids and their many functional roles within the cell. In addition, we highlight the role of sphingolipids in several pathologies, including inflammatory disease, cystic fibrosis, cancer, Alzheimer's and Parkinson's disease, and lysosomal storage disorders.
鞘脂是一类特殊的脂质，对许多细胞类型的质膜组成至关重要；然而，它们主要位于神经系统内。鞘脂的两亲性使其能够参与多种复杂的代谢途径。神经酰胺是所有鞘脂衍生物的结构单元，构成了一类复杂的脂质。这些脂质的生物合成和分解代谢在小范围和大范围的身体功能中起着重要作用，包括参与膜域和信号转导；细胞增殖、死亡、迁移和侵袭；炎症；以及中枢神经系统发育。最近，鞘脂已成为医学和生物学科学中几个研究领域的关注焦点，因为这些生物活性脂质已被确定为有效的信号和信使分子。鞘脂现在被用作几种病理学的治疗靶点。在这里，我们全面回顾了鞘脂的结构和代谢及其在细胞中的许多功能作用。此外，我们还强调了鞘脂在几种病理学中的作用，包括炎症性疾病、囊性纤维化、癌症、阿尔茨海默病和帕金森病以及溶酶体贮积症。
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