Physiology of gangliosides and the role of antiganglioside antibodies in human diseases.,Cellular & Molecular Immunology

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Physiology of gangliosides and the role of antiganglioside antibodies in human diseases.
Cellular & Molecular Immunology ( IF 21.8 ) Pub Date : 2020-03-09 , DOI: 10.1038/s41423-020-0388-9
Gianni Cutillo _{1,

2} , Anna-Helena Saariaho ₁ , Seppo Meri _{1,

2,

3}

Affiliation

Gangliosides are structurally and functionally polymorphic sialic acid containing glycosphingolipids that are widely distributed in the human body. They play important roles in protecting us against immune attacks, yet they can become targets for autoimmunity and act as receptors for microbes, like the influenza viruses, and toxins, such as the cholera toxin. The expression patterns of gangliosides vary in different tissues, during different life periods, as well as in different animals. Antibodies against gangliosides (AGA) can target immune attack e.g., against neuronal cells and neutralize their complement inhibitory activity. AGAs are important especially in acquired demyelinating immune-mediated neuropathies, like Guillain-Barré syndrome (GBS) and its variant, the Miller-Fisher syndrome (MFS). They can emerge in response to different microbial agents and immunological insults. Thereby, they can be involved in a variety of diseases. In addition, antibodies against GM3 were found in the sera of patients vaccinated with Pandemrix®, who developed secondary narcolepsy, strongly supporting the autoimmune etiology of the disease.

中文翻译：

神经节苷脂的生理学和抗神经节苷脂抗体在人类疾病中的作用。

神经节苷脂是在结构和功能上具有多态性的含有鞘糖脂的唾液酸，广泛分布于人体内。它们在保护我们免受免疫攻击方面发挥着重要作用，但它们也可以成为自身免疫的目标，并充当微生物（如流感病毒）和毒素（如霍乱毒素）的受体。神经节苷脂的表达模式在不同组织、不同生命时期以及不同动物中有所不同。抗神经节苷脂（AGA）的抗体可以针对例如神经元细胞的免疫攻击并中和其补体抑制活性。AGA 在获得性脱髓鞘性免疫介导的神经病中尤其重要，例如吉兰-巴利综合征 (GBS) 及其变体米勒-费雪综合征 (MFS)。它们可以针对不同的微生物制剂和免疫损伤而出现。因此，它们可能与多种疾病有关。此外，在接种 Pandemrix® 的患者血清中发现了针对 GM3 的抗体，这些患者出现了继发性嗜睡病，这有力地支持了该疾病的自身免疫病因学。

更新日期：2020-04-24

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