Zhu Wenli, Li Ke, Cui Tingting, Yan Yaping
Key Laboratory of the Ministry of Education for Medicinal Resources and Natural Pharmaceutical Chemistry, National Engineering Laboratory for Resource Development of Endangered Crude Drugs in Northwest of China, College of Life Sciences, Shaanxi Normal University, Xi'an, China.
Ann Transl Med. 2023 Apr 15;11(7):289. doi: 10.21037/atm-20-2285. Epub 2021 Apr 21.
Gangliosides are a class of glycosphingolipid molecules that are highly enriched in cellular membranes of the nervous system. The gangliosides associated with autoimmune diseases of the nervous system are mainly GM1, GD1a, GalNAc-GD1a, GM1b, GD3, CD1b, GT1a, and GQ1b. Multiple antibodies recognizing gangliosides are associated with some acute or chronic peripheral neuropathies, especially Guillain-Barré syndrome (GBS) and its clinical variants. Antibodies binding to gangliosides can activate complement system and recruit macrophages on the axolemma at the nodes of Ranvier of motor fibers, which are found in the course of GBS, causing axonal degeneration and reversible conduction block or conduction failure. Testing of anti-gangliosides autoantibodies is helpful for diagnosis of autoimmune peripheral neuropathies or support the diagnosis of the subtypes. These anti-gangliosides antibodies are usually detected by several qualitative or quantitative methods, particularly enzyme-linked immunosorbent assay (ELISA) and immunodot assays, which have been commercialized or established in-house worldwide. Herein, we introduce the methods and clinical applications of these assays in the diagnosis of autoimmune peripheral neuropathies. Anti-gangliosides antibodies are diagnostic markers of GBS subtypes. We use GBS as an example to explain the role of anti-gangliosides antibodies in the pathogenesis and diagnostic classification of neuropathies.
神经节苷脂是一类糖鞘脂分子,在神经系统的细胞膜中高度富集。与神经系统自身免疫性疾病相关的神经节苷脂主要有GM1、GD1a、GalNAc-GD1a、GM1b、GD3、CD1b、GT1a和GQ1b。多种识别神经节苷脂的抗体与一些急性或慢性周围神经病相关,尤其是吉兰-巴雷综合征(GBS)及其临床变异型。与神经节苷脂结合的抗体可激活补体系统,并在运动纤维郎飞结的轴膜上募集巨噬细胞,这在GBS病程中可见,导致轴突变性和可逆性传导阻滞或传导衰竭。抗神经节苷脂自身抗体检测有助于自身免疫性周围神经病的诊断或支持亚型诊断。这些抗神经节苷脂抗体通常通过几种定性或定量方法检测,尤其是酶联免疫吸附测定(ELISA)和免疫斑点测定,这些方法已在全球范围内商业化或内部建立。在此,我们介绍这些检测方法及其在自身免疫性周围神经病诊断中的临床应用。抗神经节苷脂抗体是GBS亚型的诊断标志物。我们以GBS为例解释抗神经节苷脂抗体在神经病发病机制和诊断分类中的作用。