Guillain–Barré syndrome: update on immunobiology and treatment

Guillain–Barré syndrome: update on immunobiology and treatment
复制标题

格林-巴利综合征:免疫生物学和治疗的最新进展

DOI:
--
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发表时间:
2009
影响因子:
4.3
通讯作者:
S. Kusunoki
S. Kusunoki
中科院分区:
医学3区
文献类型:
--
作者:
K. Kaida;S. Kusunoki

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越来越多的实验和临床数据揭示了吉兰-巴雷综合征(GBS)的病理生理学,并进一步促进了新的治疗策略的发展。抗神经节苷脂抗体滴度升高是GBS的特征。这可以通过与靶神经节苷脂抗原定位的位点结合来确定每个病例的临床特征。在GBS及其变体的实验模型中,互补抑制剂可以通过抑制抗神经节苷脂抗体介导的经典途径的激活来发挥神经保护功效。补体介导的电压门控钠通道簇的破坏已被证明是GBS急性运动轴突变体模型中传导失败的主要原因,受补体抑制剂保护。抗GQ 1b抗体介导的运动神经末梢损伤也受到补体抑制剂的保护。在未来,许多种类的候选药物,抑制补体系统在各个阶段的激活将被用于自身免疫性神经病变的模型,在未来应用于GBS及其变体的临床试验。电压门控钙通道的补体非依赖性阻断或神经元的凋亡机制也应考虑在内。抗神经节苷脂抗体的致病作用取决于神经膜内糖脂的局部环境和抗体的特异性。
Growing experimental and clinical data have shed light on the pathophysiology of Guillain–Barré syndrome (GBS) and have further promoted the development of novel therapeutic strategies for the disorder. Elevated titer of antiganglioside antibodies is a characteristic of GBS. This may determine the clinical features of each case by binding to the sites where a target ganglioside antigen is localized. In experimental models of GBS and its variants, complementary inhibitory agents may exert neuroprotective efficacy by inhibiting antiganglioside antibody-mediated activation of the classical pathway. Complement-mediated disruption of the voltage-gated sodium channel cluster has been shown to be a principal cause of conduction failure in the model of acute motor axonal variants of GBS, protected by a complement inhibitor. Anti-GQ1b antibody-mediated injury at motor nerve terminals is also protected by complement inhibitors. In the future many kinds of drug candidates that inhibit activation of the complement system at various stages will be used in models of autoimmune neuropathy, in future applied to clinical trials for GBS and its variants. Complement-independent blockade of voltage-gated calcium channels or the apoptotic mechanism of neurons should also be considered. The pathogenic effect of antiganglioside antibody depends upon the local glycolipid environment in the nerve membrane as well as the antibody specificity.
神经节苷脂样部分在周围神经中的分布。
DOI: 10.1093/brain/122.3.449
发表时间: 1999
期刊: Brain : a journal of neurology
影响因子: --
作者:
Sheikh,KA;Deerinck,TJ;Ellisman,MH;Griffin,JW
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DOI: 10.1016/j.bbagen.2007.10.008
发表时间: 2008-03-01
影响因子: 3
作者:
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DOI: --
发表时间: 1993
期刊: The Journal of biological chemistry
影响因子: --
作者:
Norgard,KE;Moore,KL;Diaz,S;Stults,NL;Ushiyama,S;McEver,RP;Cummings,RD;Varki,A
通讯作者: Varki,A