Department of Neurology, Affiliated Hospital of Jining Medical University, Jining, Shandong Province, People's Republic of China; Central Laboratory, Affiliated Hospital of Jining Medical University, Jining, Shandong Province, People's Republic of China.
Department of Neurology, Affiliated Hospital of Jining Medical University, Jining, Shandong Province, People's Republic of China.
Exp Neurol. 2017 May;291:134-140. doi: 10.1016/j.expneurol.2017.02.010. Epub 2017 Feb 16.
Autoantibodies binding to peripheral nerves followed by complement deposition and membrane attack complex formation results in nerve damage in Guillain-Barré syndrome (GBS). Strategies to remove the pathogenic autoantibodies or block the complement deposition benefit most patients with GBS. Immunoglobulin G-degrading enzyme of Streptococcus pyogenes (IdeS) is a cysteine protease which cleaves IgG antibodies into F(ab') and Fc fragments. In this study, using a rabbit model of axonal GBS, acute motor axonal neuropathy (AMAN), we demonstrated that IdeS treatment significantly reduced the disruption of Nav channels as well as activated C3 deposition at the anterior spinal root nodes of Ranvier in AMAN rabbits. IdeS significantly promoted the clinical recovery of AMAN rabbits and there were significant lower frequencies of axonal degeneration in anterior spinal roots of AMAN rabbits with IdeS treatment compared to the saline controls. Our data support that IdeS treatment is a promising therapeutic strategy for GBS.
自身抗体与周围神经结合，随后补体沉积和膜攻击复合物形成，导致吉兰-巴雷综合征（GBS）的神经损伤。去除致病性自身抗体或阻断补体沉积的策略对大多数 GBS 患者有益。化脓性链球菌免疫球蛋白 G 降解酶（IdeS）是一种半胱氨酸蛋白酶，可将 IgG 抗体切割成 F(ab')和 Fc 片段。在这项研究中，我们使用轴索性 GBS（急性运动轴索性神经病，AMAN）的兔模型证明，IdeS 治疗可显著减少 Nav 通道的破坏，并减少 AMAN 兔Ranvier 前脊神经根处的 C3 激活沉积。IdeS 显著促进了 AMAN 兔的临床恢复，与盐水对照组相比，IdeS 治疗的 AMAN 兔前脊神经根中的轴突变性频率明显更低。我们的数据支持 IdeS 治疗是 GBS 的一种有前途的治疗策略。
