Grassi Sara, Prioni Simona, Cabitta Livia, Aureli Massimo, Sonnino Sandro, Prinetti Alessandro
Department of Medical Biotechnology and Translational Medicine, University of Milan, Via Fratelli Cervi 93, 20090, Segrate, Milan, Italy.
Neurochem Res. 2016 Feb;41(1-2):130-43. doi: 10.1007/s11064-015-1747-2. Epub 2015 Nov 5.
Sulfatide (3-O-sulfogalactosylceramide, SM4s) was isolated by Thudichum from the human brain in 1884. Together with galactosylceramide, its direct metabolic precursor in the biosynthetic pathway, sulfatide is highly enriched in myelin in the central and peripheral nervous system, and it has been implicated in several aspects of the biology of myelin-forming cells. Studies obtained using galactolipid-deficient mice strongly support the notion that sulfatide plays critical roles in the correct structure and function of myelin membrane. A number of papers are suggesting that these roles are mediated by a specific function of sulfatide in the lateral organization of myelin membrane, thus affecting the sorting, lateral assembly, membrane dynamics and also the function of specific myelin proteins in different substructures of the myelin sheath. The consequences of altered sulfatide metabolism and sulfatide-mediated myelin organization with respect to myelin diseases are still poorly understood, but it's very likely that sulfatide might represent not only a critical player in the pathogenesis of several diseases, including multiple sclerosis and Alzheimer's disease, but also a potentially promising therapeutic target.
硫苷脂（3-O-磺基半乳糖基神经酰胺，SM4s）于1884年由图迪雄从人脑中分离出来。与它在生物合成途径中的直接代谢前体半乳糖基神经酰胺一起，硫苷脂在中枢和外周神经系统的髓鞘中高度富集，并且与形成髓鞘细胞生物学的多个方面有关。使用缺乏半乳糖脂的小鼠进行的研究有力地支持了硫苷脂在髓鞘膜的正确结构和功能中起关键作用的观点。许多论文表明，这些作用是由硫苷脂在髓鞘膜侧向组织中的特定功能介导的，从而影响髓鞘鞘不同亚结构中特定髓鞘蛋白的分选、侧向组装、膜动力学以及功能。关于髓鞘疾病，硫苷脂代谢改变和硫苷脂介导的髓鞘组织的后果仍知之甚少，但硫苷脂很可能不仅是包括多发性硬化症和阿尔茨海默病在内的几种疾病发病机制中的关键因素，而且还是一个潜在的有前景的治疗靶点。
