INSERM, IMRB, Univ Paris Est Créteil, F-94010 Créteil, France.
Department of Biochemistry and Molecular & Cellular Biology, Georgetown University, Washington, DC 20007, USA.
Int J Mol Sci. 2020 Dec 17;21(24):9645. doi: 10.3390/ijms21249645.
Gangliosides constitute a subgroup of glycosphingolipids characterized by the presence of sialic acid residues in their structure. As constituents of cellular membranes, in particular of raft microdomains, they exert multiple functions, some of them capital in cell homeostasis. Their presence in cells is tightly regulated by a balanced expression and function of the enzymes responsible for their biosynthesis, ganglioside synthases, and their degradation, glycosidases. The dysregulation of their abundance results in rare and common diseases. In this review, we make a point on the relevance of gangliosides and some of their metabolic precursors, such as ceramides, in the function of podocytes, the main cellular component of the glomerular filtration barrier, as well as their implications in podocytopathies. The results presented in this review suggest the pertinence of clinical lipidomic studies targeting these metabolites.
神经节苷脂是糖鞘脂的一个亚组，其结构中存在唾液酸残基。作为细胞膜的组成部分，特别是筏微域的组成部分，它们发挥多种功能，其中一些对细胞内稳态至关重要。它们在细胞中的存在受到负责其生物合成、神经节苷脂合成酶及其降解、糖苷酶的酶的平衡表达和功能的严格调节。它们丰度的失调导致罕见和常见疾病。在这篇综述中，我们强调了神经节苷脂及其一些代谢前体（如神经酰胺）在肾小球滤过屏障主要细胞成分足细胞功能中的重要性，以及它们在足细胞病中的意义。本综述中提出的结果表明，针对这些代谢物的临床脂质组学研究具有相关性。
