Fredman P, Lekman A
Department of Psychiatry and Neurochemistry, Göteborg University, Sweden.
Neurochem Res. 1997 Aug;22(8):1071-83. doi: 10.1023/a:1022495430583.
Glycosphingolipids are most abundant in the nervous system within which are developmental, regional, structural and cellular differences regarding their composition. The are shedded to the cerebrospinal fluid and thus potential markers for pathogenic alterations in the brain, such as developmental abnormalities, demyelination, gliosis, neuronal cell destruction. The glycosphingolipids have also been found to be antigens in autoimmune processes involving the nervous system, in particular in peripheral neuropathies like Guillain Barré syndrome, multifocal motor neuropathy etc. The immune response might have been triggered by infectious agents with an antigen epitope which mimic the glycosphingolipid or by a primary nerve tissue damage leading to release of glycosphingolipids. There is a series of support for a clinical significance of cerebrospinal fluid glycosphingolipid determinations and the presence of anti-glycosphingolipid antibodies but this has to be further explored. This paper is a mini review of the state of the art and discuss methodological aspects and improvements that might help to explore the relevance of glycosphingolipids in neurological disorders.
糖鞘脂在神经系统中含量最为丰富,其组成在发育、区域、结构和细胞方面存在差异。它们会脱落到脑脊液中,因此可能是大脑致病改变的标志物,如发育异常、脱髓鞘、胶质增生、神经元细胞破坏。糖鞘脂还被发现是涉及神经系统的自身免疫过程中的抗原,特别是在格林 - 巴利综合征、多灶性运动神经病等周围神经病中。免疫反应可能是由具有模拟糖鞘脂抗原表位的感染因子触发,或者是由导致糖鞘脂释放的原发性神经组织损伤触发。有一系列证据支持脑脊液糖鞘脂测定及抗糖鞘脂抗体存在的临床意义,但这仍有待进一步探索。本文是一篇关于当前技术水平的小型综述,讨论了可能有助于探索糖鞘脂在神经系统疾病中相关性的方法学方面及改进措施。