Departments of Pharmacology and Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD, United States.
Adv Carbohydr Chem Biochem. 2019;76:113-148. doi: 10.1016/bs.accb.2018.09.002. Epub 2018 Oct 23.
Gangliosides comprise a varied family of glycosphingolipid structures bearing one or more sialic acid residues. They are found in all mammalian tissues but are most abundant in the brain, where they represent the quantitatively major class of sialoglycans. As prominent molecular determinants on cell surfaces, they function as molecular-recognition partners for diverse glycan-binding proteins ranging from bacterial toxins to endogenous cell-cell adhesion molecules. Gangliosides also regulate the activity of plasma membrane proteins, including protein tyrosine kinases, by lateral association in the same membranes in which they reside. Their roles in molecular recognition and membrane protein regulation implicate gangliosides in human physiology and pathology, including infectious diseases, diabetes, cancer, and neurodegeneration. The varied structures and biosynthetic pathways of gangliosides are presented here, along with representative examples of their biological functions in health and disease.
神经节苷脂是糖鞘脂结构的一个多样化家族，含有一个或多个唾液酸残基。它们存在于所有哺乳动物组织中，但在大脑中含量最丰富，是数量上主要的唾液酸糖蛋白聚糖。作为细胞表面的重要分子决定因素，它们作为分子识别伴侣，与从细菌毒素到内源性细胞-细胞粘附分子等多种糖结合蛋白相互作用。神经节苷脂还通过在其所在的同一膜中侧向缔合，调节质膜蛋白的活性，包括蛋白酪氨酸激酶。它们在分子识别和膜蛋白调节中的作用使神经节苷脂与人类生理学和病理学有关，包括传染病、糖尿病、癌症和神经退行性疾病。本文介绍了神经节苷脂的各种结构和生物合成途径，以及它们在健康和疾病中的生物学功能的代表性例子。
