Ledeen Robert, Wu Gusheng
Division of Neurochemistry, Department of Pharmacology, Physiology & Neuroscience, Rutgers New Jersey Medical School, Newark, NJ, USA.
Methods Mol Biol. 2018;1804:19-55. doi: 10.1007/978-1-4939-8552-4_2.
This review begins by attempting to recount some of the pioneering discoveries that first identified the presence of gangliosides in the nervous system, their structures and topography. This is presented as prelude to the current emphasis on physiological function, about which much has been learned but still remains to be elucidated. These areas include ganglioside roles in nervous system development including stem cell biology, membranes and organelles within neurons and glia, ion transport mechanisms, receptor modulation including neurotrophic factor receptors, and importantly the pathophysiological role of ganglioside aberrations in neurodegenerative disorders. This relates to their potential as therapeutic agents, especially in those conditions characterized by deficiency of one or more specific gangliosides. Finally we attempt to speculate on future directions ganglioside research is likely to take so as to capitalize on the impressive progress to date.
本综述首先尝试回顾一些开创性的发现,这些发现首次确定了神经节苷脂在神经系统中的存在、结构和分布情况。这部分内容作为当前对生理功能重点研究的前奏呈现,关于生理功能虽已了解很多,但仍有待阐明。这些领域包括神经节苷脂在神经系统发育中的作用,包括干细胞生物学、神经元和神经胶质细胞内的膜及细胞器、离子转运机制、受体调节(包括神经营养因子受体),以及重要的是神经节苷脂异常在神经退行性疾病中的病理生理作用。这涉及它们作为治疗药物的潜力,特别是在那些以一种或多种特定神经节苷脂缺乏为特征的病症中。最后,我们试图推测神经节苷脂研究未来可能的方向,以便利用迄今为止取得的显著进展。