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cytotoxicity\uff09\uff0c\u5bf9\u8fdb\u4e00\u6b65\u7406\u89e3\u75be\u75c5\u7684\u53d1\u751f\u6709\u91cd\u8981\u610f\u4e49<sup>\uff3b<\/sup><sup>9\uff3d<\/sup>\u3002<\/p>\n<p>\uff08\u4e8c\uff09AQP4\u7684\u529f\u80fd<\/p>\n<p>AQP4\u5c5e\u4e8e\u9009\u62e9\u6027\u6c34\u901a\u9053\u86cb\u767d\u5bb6\u65cf\uff0c\u662f\u4e00\u79cd\u53cc\u5411\u3001\u6e17\u900f\u9a71\u52a8\u7684\u6c34\u901a\u9053\uff0c\u662f\u5927\u8111\u3001\u810a\u9ad3\u548c\u89c6\u795e\u7ecf\u4e2d\u6700\u4e30\u5bcc\u7684\u6c34\u901a\u9053\uff0c\u901a\u8fc7\u53c2\u4e0e\u6c34\u5206\u5b50\u7a7f\u8fc7\u8840\u8111\u5c4f\u969c\u7684\u8fc7\u7a0b\u6765\u63a7\u5236\u795e\u7ecf\u7cfb\u7edf\u7684\u6c34\u7a33\u6001<sup>\uff3b<\/sup><sup>5\uff3d<\/sup>\u3002\u5173\u4e8eAQP4\u7684\u529f\u80fd\u7814\u7a76\u5927\u591a\u662f\u57fa\u4e8eAQP4\u57fa\u56e0\u7f3a\u9677\u52a8\u7269\u7684\u7814\u7a76\uff0c\u7814\u7a76\u53d1\u73b0AQP4\u53c2\u4e0e\u8111\u810a\u6db2\u7684\u4ea7\u751f\u5e76\u5728\u5185\u73af\u5883\u7a33\u5b9a\u4e2d\u53d1\u6325\u91cd\u8981\u4f5c\u7528<sup>\uff3b<\/sup><sup>10\uff3d<\/sup>\u3002\u6c34\u4e2d\u6bd2\u548c\u7f3a\u8840\u6027\u8111\u6897\u6b7b\u6a21\u578b\u4e2dAQP4\u57fa\u56e0\u7f3a\u9677\u7684\u5c0f\u9f20\u8111\u6c34\u80bf\u51cf\u8f7b<sup>\uff3b<\/sup><sup>11\uff3d<\/sup>\uff1b\u5728\u8840\u7ba1\u6e90\u6027\u6c34\u80bf\u548c\u8111\u79ef\u6c34\u6a21\u578b\u4e2dAQP4\u57fa\u56e0\u7f3a\u9677\u5c0f\u9f20\u7684\u9884\u540e\u66f4\u5dee<sup>\uff3b<\/sup><sup>12\uff3d<\/sup>\uff0c\u8fd9\u4e9b\u53d1\u73b0\u8868\u660e\u4e86AQP4\u5728\u8111\u4e2d\u5bf9\u6c34\u5e73\u8861\u8c03\u63a7\u7684\u91cd\u8981\u4f5c\u7528\u3002<\/p>\n<p>\u6b64\u5916\uff0c\u8d8a\u6765\u8d8a\u591a\u7684\u7814\u7a76\u7ed3\u679c\u8868\u660e\uff0cAQP4\u8fd8\u53c2\u4e0e\u5176\u4ed6\u5404\u79cd\u751f\u7406\u529f\u80fd\uff0c\u5982\u795e\u7ecf\u5174\u594b\u3001\u7a81\u89e6\u53ef\u5851\u6027\u548c\u8bb0\u5fc6\u5f62\u6210\u3001\u6dcb\u5df4\u6db2\u6e05\u9664\u3001\u795e\u7ecf\u708e\u75c7\u3001\u8c37\u6c28\u9178\u7a33\u6001\u3001\u94be\u79bb\u5b50\u5e73\u8861\u3001\u7ec6\u80de\u5916\u7a7a\u95f4\u4f53\u79ef\u8c03\u8282\u7b49<sup>\uff3b<\/sup><sup>13\uff3d<\/sup>\u3002<\/p>\n<p>\uff08\u4e09\uff09AQP4\u7684\u5206\u5e03<\/p>\n<p>AQP4\u4e3b\u8981\u5728CNS\u7684\u661f\u5f62\u80f6\u8d28\u7ec6\u80de\u3001\u5ba4\u7ba1\u819c\u7ec6\u80de\u548c\u89c6\u7f51\u819cM\u00fcller\u7ec6\u80de\u4e2d\u8868\u8fbe\uff0c\u4f46\u4e0d\u5b58\u5728\u4e8e\u5c11\u7a81\u80f6\u8d28\u7ec6\u80de\u3001\u795e\u7ecf\u5143\u6216\u8109\u7edc\u819c\u4e0a\u76ae\u7ec6\u80de\u3002\u5728\u8840\u7ba1\u9644\u8fd1\u7684\u661f\u5f62\u80f6\u8d28\u7ec6\u80de\u672b\u7aef\u8868\u8fbe\u6700\u591a\uff0c\u4e0e\u8840\u8111\u5c4f\u969c\u63a5\u89e6\uff0c\u8fd9\u79cd\u805a\u96c6\u8868\u8fbe\u4e5f\u79f0\u4e3aAQP4\u7684\u6781\u5316\u8868\u8fbe\u3002AQP4\u8fd8\u5728\u80be\u3001\u6c14\u9053\u3001\u80c3\u80a0\u5668\u5b98\u7684\u4e0a\u76ae\u7ec6\u80de\u57fa\u5e95\u5916\u4fa7\u819c\u8868\u8fbe\uff0c\u5728\u9aa8\u9abc\u808c\u4e2d\u8868\u8fbe\u6c34\u5e73\u8f83\u4f4e<sup>\uff3b<\/sup><sup>6\uff3d<\/sup>\u3002\u867d\u7136AQP4\u4e5f\u5728\u8fd9\u4e9b\u5916\u5468\u5668\u5b98\u4e2d\u5e7f\u6cdb\u8868\u8fbe\uff0c\u4f46\u5e76\u672a\u51fa\u73b0\u75c5\u7406\u6539\u53d8\uff0c\u8fd9\u53ef\u80fd\u662f\u7531\u4e8e\u8865\u4f53\u6fc0\u6d3b\u8c03\u8282\u56e0\u5b50\u5728\u5916\u5468\u4e0eAQP4\u5171\u8868\u8fbe\u6bd4CNS\u66f4\u9ad8<sup>\uff3b<\/sup><sup>14\uff3d<\/sup>\u3002AQP4\u7684\u5206\u5e03\u4e0eAQP4-IgG\u6297\u4f53\u9633\u6027\u60a3\u8005\u7684\u75c5\u53d8\u5206\u5e03\u9ad8\u5ea6\u76f8\u5173\uff0c\u7279\u5f81\u6027\u75c5\u7076\u4f4d\u4e8e\u89c6\u795e\u7ecf\u3001\u810a\u9ad3\u3001\u95f4\u8111\u548c\u540e\u8111\u533a\uff0c\u76f8\u5e94\u90e8\u4f4d\u53d7\u7d2f\u4fbf\u53ef\u51fa\u73b0\u5bf9\u5e94\u75c7\u72b6\u3002You\u7b49<sup>\uff3b<\/sup><sup>15\uff3d<\/sup>\u5bf9\u4eba\u89c6\u7f51\u819c\u4e2d\u7684AQP4\u548cM\u00fcller\u7ec6\u80de\u7684\u529f\u80fd\u7d0a\u4e71\u8fdb\u884c\u4e86\u8be6\u7ec6\u5206\u6790\uff0c\u63d0\u4f9b\u4e86AQP4-IgG\u9633\u6027\u60a3\u8005\u773c\u90e8M\u00fcller\u7ec6\u80de\u529f\u80fd\u969c\u788d\u7684\u4f53\u5185\u7ed3\u6784\u548c\u529f\u80fd\u8bc1\u636e\u3002<\/p>\n<p>\u6709\u7814\u7a76\u7ed3\u679c\u8868\u660e\uff0cAQP4\u7684\u6781\u5316\u8868\u8fbe\u53ef\u80fd\u4e0e\u5176C\u7aef\u4e1d\u6c28\u9178-\u4e1d\u6c28\u9178-\u7f2c\u6c28\u9178\uff08Ser-Ser-Val\uff09\u5e8f\u5217\u6709\u5173\uff0c\u8fd9\u6bb5\u5e8f\u5217\u662fPDZ\u7ed3\u6784\u57df\uff08postsynaptic density protein-95\uff0cdiscs large\uff0cand zona occludens 1 proteins\uff09\u7684\u7279\u5f81\u6027\u7ed3\u5408\u57fa\u5e8f\uff0c\u6b64\u5916\u805a\u96c6\u86cb\u767d\u3001\u808c\u8425\u517b\u4e0d\u826f\u86cb\u767d\u76f8\u5173\u86cb\u767d\u590d\u5408\u7269\u4ee5\u53ca\u661f\u5f62\u80f6\u8d28\u7ec6\u80de\u4e0e\u5185\u76ae\u7ec6\u80de\u548c\u8f6f\u8111\u819c\u7ec6\u80de\u7684\u63a5\u89e6\u5bf9AQP4\u6781\u5316\u4e5f\u6709\u91cd\u8981\u4f5c\u7528<sup>\uff3b<\/sup><sup>16\uff3d<\/sup>\u3002<\/p>\n<p>\u4e8c\u3001AQP4-IgG\u4e0eNMOSD<\/p>\n<p>\uff08\u4e00\uff09AQP4-IgG\u7684\u4ea7\u751f<\/p>\n<p>\u73b0\u9636\u6bb5\u5bf9\u4e8eNMOSD\u60a3\u8005\u4ea7\u751f\u81ea\u8eab\u7279\u5f02\u6027AQP4-IgG\u7684\u673a\u5236\u5c1a\u672a\u660e\u786e\uff0c\u53ef\u80fd\u7684\u539f\u56e0\u6709\u4ee5\u4e0b\u51e0\u70b9\uff1a\uff081\uff09\u514d\u75ab\u8010\u53d7\u7f3a\u9677\uff1a\u7814\u7a76\u53d1\u73b0\u5728NMOSD\u60a3\u8005\u7684\u4e2d\u67a2\u548c\u5916\u5468B\u7ec6\u80de\u8010\u53d7\u68c0\u67e5\u70b9\u5b58\u5728\u7f3a\u9677\uff0c\u5bfc\u81f4\u81ea\u8eab\u53cd\u5e94\u6027\u5e7c\u7a1aB\u7ec6\u80de\u8fc7\u591a\uff0c\u81ea\u8eab\u53cd\u5e94\u6027B\u7ec6\u80de\u4e0d\u80fd\u88ab\u6e05\u9664\u6216\u4f7f\u5176\u5904\u4e8e\u65e0\u53cd\u5e94\u72b6\u6001\uff0c\u5bfc\u81f4\u5728\u5176\u5e7c\u7a1aB\u7ec6\u80de\u79ef\u7d2f\uff0c\u4f34\u968f\u5927\u91cf\u81ea\u8eab\u53cd\u5e94\u6027\u514b\u9686\uff0c\u514d\u75ab\u8010\u53d7\u7f3a\u9677\u53ef\u80fd\u662fAQP4\u81ea\u8eab\u6297\u4f53\u4ea7\u751f\u548c\u968f\u540e\u75be\u75c5\u53d1\u751f\u7684\u5173\u952e\u56e0\u7d20\u4e4b\u4e00<sup>\uff3b<\/sup><sup>17\uff3d<\/sup>\u3002\uff082\uff09\u5206\u5b50\u6a21\u62df\uff1a\u6709\u7814\u7a76\u53d1\u73b0\uff0c\u81ea\u7136\u754c\u4e2d\u7684\u51e0\u79cd\u690d\u7269\u548c\u7ec6\u83cc\u86cb\u767d\u8d28\u5728\u5e8f\u5217\u548c\u7ed3\u6784\u4e0a\u4e0e\u4eba\u7c7bAQP4\u6709\u663e\u8457\u7684\u76f8\u4f3c\u6027<sup>\uff3b<\/sup><sup>18\uff3d<\/sup>\u3002\u53e6\u6709\u5b66\u8005\u53d1\u73b0\u4e0eAQP4\u53cd\u5e94\u7684T\u7ec6\u80de\u4f1a\u4e0e\u4ea7\u6c14\u835a\u819c\u68ad\u83cc\u86cb\u767d\u8d28\u5185\u7684\u540c\u6e90\u80bd\u5e8f\u5217\u8868\u73b0\u51fa\u4ea4\u53c9\u53cd\u5e94<sup>\uff3b<\/sup><sup>19\uff3d<\/sup>\u3002\u8fd9\u4e9b\u7814\u7a76\u7ed3\u679c\u53ef\u80fd\u5bf9\u81f4\u75c5\u6027\u6297\u4f53\u7684\u4ea7\u751f\u673a\u5236\u6709\u4e00\u5b9a\u63d0\u793a\u4f5c\u7528\u3002\u6b64\u5916\uff0cIL-6\u6709\u4fc3\u8fdb\u6d46\u7ec6\u80de\u5b58\u6d3b\u3001\u589e\u5f3a\u4fc3\u708e\u6027T\u7ec6\u80de\u5206\u5316\u548c\u6fc0\u6d3b\u53ca\u523a\u6fc0AQP-IgG\u4ea7\u751f\u7b49\u4f5c\u7528\uff0c\u5728\u81f4\u75c5\u6297\u4f53\u4ea7\u751f\u53ca\u81f4\u75c5\u8fc7\u7a0b\u4e2d\u53d1\u6325\u91cd\u8981\u4f5c\u7528<sup>\uff3b<\/sup><sup>20\uff3d<\/sup>\u3002<\/p>\n<p>\u9488\u5bf9\u6297\u4f53\u7684\u4ea7\u751f\uff0c\u76ee\u524d\u6709\u5b66\u8005\u7814\u7a76\u4e86\u8bb8\u591a\u4e0d\u540c\u7684\u6cbb\u7597\u9776\u70b9\uff0c\u5305\u62ecB\u7ec6\u80de\u6297\u539fCD19\uff08inebilizumab\uff09\u3001CD20\uff08rituximab\uff09\u4ee5\u53caIL-6R\uff08satralizumab\uff09\u7b49\u9776\u70b9\u7684\u5355\u514b\u9686\u6297\u4f53\u3002inebilizumab\u548crituximab\u901a\u8fc7\u8017\u7aedB\u7ec6\u80de\u4ece\u800c\u51cf\u5c11\u81f4\u75c5\u6027\u6297\u4f53\uff0c\u5728\u4e34\u5e8a\u4e0a\u90fd\u53d6\u5f97\u4e86\u8f83\u597d\u7684\u7597\u6548<sup>\uff3b<\/sup><sup>21\uff3d<\/sup>\u3002satralizumab\u901a\u8fc7\u963b\u65adIL-6\u7684\u4f5c\u7528\uff0c\u51cf\u5c11\u81f4\u75c5\u6027\u6297\u4f53\u4ea7\u751f\uff0c\u53ef\u4ee5\u663e\u8457\u51cf\u5c11\u60a3\u8005\u75be\u75c5\u7684\u590d\u53d1<sup>\uff3b<\/sup><sup>22\uff3d<\/sup>\u3002\u6b64\u5916\uff0c\u8bf1\u5bfc\u514d\u75ab\u8010\u53d7\u662f\u4e00\u79cd\u65b0\u5174\u6280\u672f\uff0c\u6216\u8bb8\u53ef\u4ee5\u5728\u4fdd\u7559\u673a\u4f53\u6b63\u5e38\u9632\u5fa1\u673a\u5236\u7684\u540c\u65f6\u6cbb\u7597NMOSD\u3002\u4e00\u9879\u2160b\u671f\u4e34\u5e8a\u8bd5\u9a8c\u7ed3\u679c\u8868\u660e\uff0c\u4f7f\u7528\u8d1f\u8f7d\u7279\u5f02\u6027\u6297\u539f\u7684\u5de5\u7a0b\u6811\u7a81\u72b6\u7ec6\u80de\u8bf1\u5bfc\u8010\u53d7\u662f\u5b89\u5168\u53ef\u884c\u7684<sup>\uff3b<\/sup><sup>23\uff3d<\/sup>\u3002<\/p>\n<p>\uff08\u4e8c\uff09AQP4-IgG\u4e0e\u75be\u75c5\u7684\u5173\u7cfb<\/p>\n<p>\u81ea\u4eceLennon\u7b49<sup>\uff3b<\/sup><sup>2\uff3d<\/sup>\u53d1\u73b0NMO\u60a3\u8005\u4e2d\u7279\u5f02\u6027\u7684AQP4-IgG\u4ee5\u6765\uff0c\u4eba\u4eec\u8ba4\u8bc6\u5230NMO\u662f\u4e00\u79cd\u4e0e\u591a\u53d1\u6027\u786c\u5316\uff08multiple sclerosis\uff0cMS\uff09\u4e0d\u540c\u7684\u72ec\u7acb\u75be\u75c5\u3002\u968f\u540e\uff0cLopez\u7b49<sup>\uff3b<\/sup><sup>24\uff3d<\/sup>\u53d1\u73b0\u5728\u90e8\u5206AQP4\u6297\u4f53\u9634\u6027\u7684NMOSD\u60a3\u8005\u4e2d\u5b58\u5728\u9ad3\u9798\u5c11\u7a81\u80f6\u8d28\u7ec6\u80de\u7cd6\u86cb\u767d\uff08myelinoligodendrocyte glycoprotein\uff0cMOG\uff09\u6297\u4f53\uff0c\u4e8e\u662f\u8fd9\u7c7b\u9ad3\u9798\u5c11\u7a81\u80f6\u8d28\u7ec6\u80de\u7cd6\u86cb\u767d\u6297\u4f53\u76f8\u5173\u6027\u75be\u75c5\uff08myelin oligodendrocyte glycoprotein antibody-associated disease\uff0cMOGAD\uff09\u7684\u60a3\u8005\u53ef\u4f5c\u4e3a\u4e00\u7c7b\u72ec\u7acb\u75be\u75c5\u5b9e\u4f53\u4e0eNMOSD\u8fdb\u884c\u533a\u5206\uff0c\u4f46\u76ee\u524d\u5bf9\u4e8eMS\u5e76\u672a\u53d1\u73b0\u6709\u7279\u5f02\u6027\u7684\u81ea\u8eab\u6297\u4f53\u3002\u4e09\u8005\u5728\u4e34\u5e8a\u8868\u73b0\u4e0a\u975e\u5e38\u76f8\u4f3c\uff0c\u6545\u8840\u6e05\u6297\u4f53\u68c0\u6d4b\u662f\u5176\u91cd\u8981\u7684\u9274\u522b\u624b\u6bb5\u3002<\/p>\n<p>\u76ee\u524d\u8ba4\u4e3aAQP4-IgG\u662fNMOSD\u7684\u4e3b\u8981\u81f4\u75c5\u56e0\u7d20\uff0c\u68c0\u6d4b\u8840\u6e05\u4e2dAQP4-IgG\u662f\u5426\u9633\u6027\u662fNMOSD\u8bca\u65ad\u7684\u6700\u91cd\u8981\u7684\u65b9\u6cd5<sup>\uff3b<\/sup><sup>25\uff3d<\/sup>\u3002\u4f46\u662fAQP4-IgG\u6ef4\u5ea6\u5728\u9884\u6d4b\u60a3\u8005\u75be\u75c5\u590d\u53d1\u4e0a\u5e76\u4e0d\u51c6\u786e\uff0c\u6709\u4e9b\u7814\u7a76\u7ed3\u679c\u8868\u660e\u5927\u591a\u6570\u60a3\u8005\u590d\u53d1\u524dAQP4-IgG\u6ef4\u5ea6\u6709\u4e0d\u540c\u7a0b\u5ea6\u7684\u589e\u52a0\uff0c\u4f46\u4e5f\u6709AQP4-IgG\u6ef4\u5ea6\u964d\u4f4e\u60a3\u8005\u590d\u53d1\u7684\u4f8b\u5b50\u3002\u968f\u7740\u65f6\u95f4\u7684\u63a8\u79fb\uff0c\u4e2a\u4f53\u95f4\u548c\u4e2a\u4f53\u5185AQP4-IgG\u7684\u7edd\u5bf9\u6c34\u5e73\u90fd\u6709\u6240\u4e0d\u540c\uff0c\u4e0d\u6613\u786e\u5b9a\u9884\u6d4b\u590d\u53d1\u7684\u6297\u4f53\u6ef4\u5ea6\u4e34\u754c\u503c<sup>\uff3b<\/sup><sup>26\uff3d<\/sup>\u3002\u6b64\u5916\uff0c\u8840\u6e05AQP4-IgG\u6ef4\u5ea6\u901a\u5e38\u4e0e\u75be\u75c5\u4e25\u91cd\u7a0b\u5ea6\u5448\u6b63\u76f8\u5173\uff0c\u4f46\u5e76\u4e0d\u662f\u6240\u6709\u8840\u6e05AQP4-IgG\u6ef4\u5ea6\u9ad8\u7684\u60a3\u8005\u4e34\u5e8a\u8868\u73b0\u90fd\u66f4\u4e25\u91cd<sup>\uff3b<\/sup><sup>27\uff3d<\/sup>\uff0c\u8fd9\u53ef\u80fd\u9884\u793a\u7740\u8fd8\u6709\u66f4\u52a0\u590d\u6742\u7684\u81f4\u75c5\u673a\u5236\u5728NMOSD\u4e2d\u53d1\u6325\u91cd\u8981\u4f5c\u7528\u3002\u5173\u4e8e\u6297\u4f53\u6ef4\u5ea6\u4e0e\u75be\u75c5\u590d\u53d1\u8fdb\u5c55\u7b49\u65b9\u9762\u7684\u5173\u7cfb\u8fd8\u6709\u5f85\u8fdb\u4e00\u6b65\u7684\u7814\u7a76\u3002<\/p>\n<p>\u4e09\u3001AQP4-IgG\u8fdb\u5165CNS<\/p>\n<p>\uff08\u4e00\uff09AQP4-IgG\u7531\u5916\u5468\u8fdb\u5165\u4e2d\u67a2<\/p>\n<p>\u76ee\u524d\u8ba4\u4e3aAQP4-IgG\u4e3b\u8981\u5b58\u5728\u4e8e\u5916\u5468\u5faa\u73af\uff0c\u901a\u8fc7\u8840\u8111\u5c4f\u969c\u5230\u8fbeCNS\u4e0e\u661f\u578b\u80f6\u8d28\u7ec6\u80de\u4e0a\u7684AQP4\u7ed3\u5408\u662f\u5bfc\u81f4\u75be\u75c5\u53d1\u751f\u7684\u4e00\u4e2a\u5173\u952e\u6b65\u9aa4\u3002<\/p>\n<p>\u4e00\u822c\u5728\u8840\u8111\u5c4f\u969c\u7684\u4f5c\u7528\u4e0b\uff0c\u8840\u6db2\u4e2d\u7684\u514d\u75ab\u7403\u86cb\u767d\u4e0d\u5bb9\u6613\u8fdb\u5165\u5230CNS\u3002\u8bb8\u591a\u7814\u7a76\u53d1\u73b0NMOSD\u90fd\u5b58\u5728\u4e0d\u540c\u7a0b\u5ea6\u7684\u8840\u8111\u5c4f\u969c\u635f\u4f24\uff0cAQP4-IgG\u53ef\u80fd\u901a\u8fc7\u5df2\u635f\u4f24\u7684\u8840\u8111\u5c4f\u969c\u8fdb\u5165CNS\uff0c\u800cAQP4-IgG\u8fdb\u5165CNS\u4e0eAQP4\u7ed3\u5408\u53ef\u5bfc\u81f4AQP4\u5185\u5316\uff0c\u6270\u4e71\u6c34\u7a33\u6001\u5e76\u5f15\u53d1\u6c34\u80bf\uff0c\u7834\u574f\u8840\u8111\u5c4f\u969c\u7684\u5b8c\u6574\u6027<sup>\uff3b<\/sup><sup>28\uff3d<\/sup>\u3002\u6b64\u5916\uff0c\u75be\u75c5\u8fc7\u7a0b\u4e2d\u81ea\u8eab\u6297\u4f53\u3001\u514d\u75ab\u7ec6\u80de\u4ee5\u53ca\u7ec6\u80de\u56e0\u5b50\u90fd\u53ef\u80fd\u635f\u5bb3\u8840\u8111\u5c4f\u969c\uff0c\u589e\u52a0\u8840\u8111\u5c4f\u969c\u7684\u901a\u900f\u6027\uff0c\u4ece\u800c\u5141\u8bb8\u66f4\u591a\u81ea\u8eab\u6297\u4f53\u8fdb\u5165CNS\uff0c\u5bfc\u81f4\u75be\u75c5\u8fdb\u4e00\u6b65\u52a0\u91cd\uff0c\u8fdb\u800c\u5bfc\u81f4\u6076\u6027\u5faa\u73af\u3002\u6709\u5b66\u8005\u53d1\u73b0IL-6\u4e0d\u4ec5\u53ef\u4ee5\u4fc3\u8fdbAQP4-IgG\u7684\u4ea7\u751f\u8fd8\u53ef\u4ee5\u8bf1\u5bfcT\u7ec6\u80de\u8fc1\u79fb\u5e76\u7834\u574f\u8840\u8111\u5c4f\u969c\u7684\u5c4f\u969c\u529f\u80fd<sup>\uff3b<\/sup><sup>29\uff3d<\/sup>\uff0c\u800c\u5728AQP4-IgG\u5b58\u5728\u7684\u6761\u4ef6\u4e0b\uff0c\u8428\u7279\u5229\u73e0\u5355\u6297\uff08satralizumab\uff09\u53ef\u4ee5\u901a\u8fc7\u8840\u8111\u5c4f\u969c\u6291\u5236\u8840\u8111\u5c4f\u969c\u529f\u80fd\u969c\u788d\u548c\u708e\u6027\u7ec6\u80de\u6d78\u6da6\uff0c\u4ece\u800c\u9884\u9632\u75be\u75c5\u7684\u53d1\u751f\u3002<\/p>\n<p>\u4e5f\u6709\u7814\u7a76\u53d1\u73b0\u8840\u8111\u5c4f\u969c\u7684\u635f\u4f24\u53ef\u80fd\u4e0d\u662f\u75c5\u53d8\u5f62\u6210\u7684\u524d\u63d0\u6761\u4ef6\uff0c\u8f6f\u8111\u819c\u7ec6\u80de\u5c42\u5728\u5c0f\u52a8\u8109\u6c34\u5e73\u4e0a\u5f00\u6709\u7a97\uff0c\u5728\u6bdb\u7ec6\u8840\u7ba1\u3001\u5c0f\u9759\u8109\u548c\u9759\u8109\u6c34\u5e73\u4e0a\u5e7f\u6cdb\u7f3a\u5931\u3002\u5728\u8fd9\u4e9b\u533a\u57df\uff0c\u8868\u8fbeAQP4\u7684\u661f\u5f62\u80f6\u8d28\u7ec6\u80de\u672b\u7aef\u76f4\u63a5\u66b4\u9732\u4e8e\u79bb\u5f00\u5fae\u8840\u7ba1\u7684\u6297\u4f53\u3002\u6b64\u5916\uff0cAQP4-IgG\u8fd8\u53ef\u4ee5\u901a\u8fc7\u8111\u5ba4\u5468\u56f4\u5668\u5b98\u7b49\u8840\u8111\u5c4f\u969c\u901a\u900f\u6027\u8f83\u5f3a\u7684\u533a\u57df\u6216\u901a\u8fc7\u7ec6\u80de\u5916\u9014\u5f84\u8fdb\u5165\u8111\u810a\u6db2<sup>\uff3b<\/sup><sup>30\uff3d<\/sup>\u3002<\/p>\n<p>\uff08\u4e8c\uff09AQP4-IgG\u9798\u5185\u5408\u6210<\/p>\n<p>\u9664\u4e86\u4ece\u5916\u5468\u5faa\u73af\u8fdb\u5165CNS\uff0cAQP4-IgG\u8fd8\u53ef\u80fd\u6765\u6e90\u4e8e\u9798\u5185\u5408\u6210\u3002Bennett\u7b49<sup>\uff3b<\/sup><sup>31\uff3d<\/sup>\u901a\u8fc7\u68c0\u6d4b\u9996\u6b21\u4e34\u5e8a\u53d1\u4f5c\u7684NMO\u60a3\u8005\u8111\u810a\u6db2\u4e2d\u8fc7\u5ea6\u8868\u8fbe\u7684\u6d46\u7ec6\u80de\u514d\u75ab\u7403\u86cb\u767d\u5e8f\u5217\uff0c\u53d1\u73b0AQP4-IgG\u5728\u53d1\u75c5\u65f6\u5b58\u5728\u9798\u5185\u5408\u6210\uff0c\u5e76\u76f4\u63a5\u53c2\u4e0e\u5230NMO\u7684\u53d1\u75c5\u8fc7\u7a0b\u3002Kowarik\u7b49<sup>\uff3b<\/sup><sup>32\uff3d<\/sup>\u901a\u8fc7\u6bd4\u8f83AQP4\u6297\u4f53\u9633\u6027\u60a3\u8005\u7684\u8840\u6e05\u4e0e\u8111\u810a\u6db2\u7684\u8f6c\u5f55\u7ec4\u548c\u514d\u75ab\u7403\u86cb\u767d\u7ec4\uff0c\u53d1\u73b0\u5728NMO\u6076\u5316\u671f\u95f4\uff0c\u9798\u5185\u514d\u75ab\u7403\u86cb\u767d\u5927\u90e8\u5206\u662f\u7531\u9798\u5185B\u7ec6\u80de\u4ea7\u751f\uff0c\u8fd9\u7fa4\u7ec6\u80de\u7531\u65b0\u4ea7\u751f\u7684\u514b\u9686\u548c\u5916\u5468\u6765\u6e90\u7684\u514b\u9686\u7ec4\u6210\uff0c\u53ef\u80fd\u5bfc\u81f4 NMO\u75be\u75c5\u7684\u6076\u5316\u548c\u75c5\u53d8\u5f62\u6210\uff0c\u5e76\u4e14\u53ef\u80fd\u662f\u9884\u9632\u6027\u6cbb\u7597\u7684\u91cd\u8981\u76ee\u6807\u3002<\/p>\n<p>\u56db\u3001AQP4-IgG \u5bfc\u81f4\u7ec6\u80de\u635f\u5bb3\u7684\u673a\u5236<\/p>\n<p>AQP4-IgG\u9633\u6027\u60a3\u8005CNS\u75c5\u53d8\u7684\u7279\u5f81\u662f\u8840\u7ba1\u4e2d\u5fc3\u6027IgG\u548cIgM\u6c89\u79ef\uff0cAQP4-IgG\u4e0e\u6297\u539f\u7ed3\u5408\u89e6\u53d1\u7ecf\u5178\u8865\u4f53\u7ea7\u8054\u53cd\u5e94\uff0c\u6fc0\u6d3b\u7684\u8865\u4f53\u4ee5\u53ca\u661f\u5f62\u80f6\u8d28\u7ec6\u80de\u5206\u6ccc\u7684\u7ec6\u80de\u56e0\u5b50\u8bf1\u5bfc\u708e\u6027\u7ec6\u80de\u6d78\u6da6\uff0c\u968f\u540e\u5f15\u8d77\u7684CNS\u5174\u594b\u6027\u6bd2\u6027\u4e0e\u65c1\u89c2\u8005\u6548\u5e94\uff0c\u9020\u6210\u8131\u9ad3\u9798\u4e0e\u795e\u7ecf\u635f\u4f24\u3002MS\u4e0eMOGAD\u7684\u75c5\u7406\u5b66\u7279\u70b9\u662f\u9759\u8109\u5468\u56f4\u6c47\u5408\u6027\u8131\u9ad3\u9798\u3001\u90e8\u5206\u8f74\u7a81\u4fdd\u7559\u4ee5\u53ca\u767d\u8d28\u548c\u7070\u8d28\u4e2d\u7684\u53cd\u5e94\u6027\u80f6\u8d28\u589e\u751f\u3002\u5728NMOSD\u4e2d\uff0c\u8f74\u7a81\u548c\u661f\u5f62\u80f6\u8d28\u7ec6\u80de\u7684\u4e22\u5931\u975e\u5e38\u4e25\u91cd\uff0c\u800c\u5728MS\u548cMOGAD\u4e2d\u661f\u5f62\u80f6\u8d28\u7ec6\u80de\u548c\u8f74\u7a81\u5927\u91cf\u4fdd\u7559\u3002\u5728NMOSD\u4e2d\u89c2\u5bdf\u5230AQP4\u4e0b\u8c03\uff0c\u800c\u5728MOGAD\u4e2dAQP4\u4fdd\u7559\u751a\u81f3\u5347\u9ad8<sup>\uff3b<\/sup><sup>24\uff3d<\/sup>\u3002<\/p>\n<p>\uff08\u4e00\uff09\u81f4\u75c5\u6027\u6297\u4f53\u7684\u4f5c\u7528<\/p>\n<p>\u7edd\u5927\u591a\u6570AQP4-IgG\u90fd\u662fIgG1\uff0c\u800cIgG1\u53ef\u6709\u6548\u6fc0\u6d3b\u8865\u4f53\u7cfb\u7edf\uff0c\u5f15\u53d1\u8865\u4f53\u4f9d\u8d56\u7684\u7ec6\u80de\u6bd2\u6027<sup>\uff3b<\/sup><sup>33\uff3d<\/sup>\u3002AQP4-IgG\u4e0eOAPs\u7ed3\u5408\u540e\uff0c\u653b\u819c\u590d\u5408\u7269\uff08membrane attack complex\uff0cMAC\uff09\u901a\u8fc7\u7834\u574f\u5c40\u90e8\u78f7\u8102\u53cc\u5206\u5b50\u5c42\u5f62\u6210\u7a7f\u819c\u4eb2\u6c34\u5b54\u9053\uff0c\u7ec6\u80de\u5438\u6c34\u80bf\u80c0\u7834\u88c2\u3002\u6b64\u8fc7\u7a0b\u4e2d\u4ea7\u751f\u7684\u8865\u4f53\u6d3b\u5316\u5355\u4f4dC3a\u3001C5a\u7b49\u662f\u91cd\u8981\u7684\u708e\u6027\u4ecb\u8d28\uff0c\u53ef\u4fc3\u8fdb\u708e\u6027\u7ec6\u80de\u6d78\u6da6\u3002\u8865\u4f53\u6fc0\u6d3b\u4ea7\u751f\u7684C3b\u3001C4b\u7b49\u8865\u4f53\u6210\u5206\u7ed3\u5408\u5728\u9776\u7ec6\u80de\u819c\u4e0a\uff0c\u901a\u8fc7\u4e0e\u541e\u566c\u7ec6\u80de\u8868\u9762\u8865\u4f53\u53d7\u4f53\u7ed3\u5408\u4fc3\u8fdb\u541e\u566c\u7ec6\u80de\u5bf9\u5176\u8fdb\u884c\u541e\u566c\uff0c\u53d1\u6325\u8c03\u7406\u4f5c\u7528<sup>\uff3b<\/sup><sup>34\uff3d<\/sup>\u3002\u6b64\u5916\uff0c\u8868\u8fbeIgG Fc\u53d7\u4f53\u7684\u81ea\u7136\u6740\u4f24\u7ec6\u80de\uff08natural killer cell\uff0cNK\uff09\u53ef\u4ee5\u901a\u8fc7\u4e0e\u5df2\u7ed3\u5408\u5728\u7ec6\u80de\u4e0a\u7684AQP4-IgG\u7684Fc\u6bb5\u7ed3\u5408\uff0c\u4ece\u800c\u6740\u4f24\u9776\u7ec6\u80de\uff0c\u53d1\u6325\u6297\u4f53\u4f9d\u8d56\u7684\u7ec6\u80de\u4ecb\u5bfc\u7684\u7ec6\u80de\u6bd2\u6027\u4f5c\u7528\uff08antibody-dependent cell-mediated cytotoxicity\uff0cADCC\uff09<sup>\uff3b<\/sup><sup>35\uff3d<\/sup>\u3002<\/p>\n<p>\u6709\u7814\u7a76\u53d1\u73b0\u6297\u4eba\u7c7b\u8865\u4f53\u86cb\u767dC1q\u5355\u514b\u9686\u6297\u4f53\u5728\u7ec6\u80de\u57f9\u517b\u548c\u810a\u9ad3\u5207\u7247\u57f9\u517b\u4e2d\u5747\u53ef\u8d77\u5230\u51cf\u8f7b\u6297\u4f53\u5f15\u8d77\u7684\u8865\u4f53\u4f9d\u8d56\u7684\u7ec6\u80de\u6bd2\u6027\uff0c\u5728NMO\u52a8\u7269\u6a21\u578b\u4e2d\u4e5f\u53ef\u89c2\u5bdf\u5230\u8131\u9ad3\u9798\u635f\u4f24\u51cf\u8f7b<sup>\uff3b<\/sup><sup>36\uff3d<\/sup>\u3002\u6700\u8fd1\u4e00\u9879\u968f\u673a\u53cc\u76f23\u671f\u4e34\u5e8a\u7814\u7a76\u4f7f\u7528C5\u6291\u5236\u5242\uff08eculizumab\uff09\u963b\u65ad\u8865\u4f53\u6fc0\u6d3b\u6765\u6cbb\u7597\u60a3\u8005\uff0c\u7ed3\u679c\u663e\u793aeculizumab\u5355\u4e00\u7597\u6cd5\u53ef\u4ee5\u957f\u671f\u6709\u6548\u5730\u9884\u9632\u75be\u75c5\u590d\u53d1<sup>\uff3b<\/sup><sup>37\uff3d<\/sup>\u3002<\/p>\n<p>\u7c7b\u4f3c\u5730\uff0c\u5927\u591a\u6570MOG\u6297\u4f53\u4e5f\u662fIgG1\uff0c\u7ed3\u5408\u5c11\u7a81\u80f6\u8d28\u7ec6\u80de\u4e0a\u7684MOG\uff0c\u7406\u8bba\u4e0a\u540c\u6837\u53ef\u4ee5\u6fc0\u6d3b\u8865\u4f53\u7cfb\u7edf\u542f\u52a8ADCC\u5bfc\u81f4\u8131\u9ad3\u9798\u3002Serguera\u7b49<sup>\uff3b<\/sup><sup>38\uff3d<\/sup>\u5728\u6052\u6cb3\u7334\u8111\u4e2d\u4e5f\u53d1\u73b0\u4e86IgG\u548cC1q\u7684\u6c89\u79ef\u3002<\/p>\n<p>\uff08\u4e8c\uff09\u708e\u6027\u7ec6\u80de\u7684\u4f5c\u7528<\/p>\n<p>\u8865\u4f53\u6d3b\u6027\u6210\u5206\u4ee5\u53ca\u7ec6\u80de\u56e0\u5b50\u53ef\u4fc3\u8fdb\u708e\u6027\u7ec6\u80de\u6d78\u6da6\uff0c\u4e3b\u8981\u5305\u62ec\u4e2d\u6027\u7c92\u7ec6\u80de\u3001\u55dc\u9178\u6027\u7c92\u7ec6\u80de\u3001\u5de8\u566c\u7ec6\u80de\/\u5c0f\u80f6\u8d28\u7ec6\u80de\u3001\u6dcb\u5df4\u7ec6\u80de<sup>\uff3b<\/sup><sup>39\uff3d<\/sup>\u3002\u503c\u5f97\u5173\u6ce8\u7684\u662f\uff0c\u5728MOGAD\u548cNMOSD\u4e2d\uff0cCD4<sup>+<\/sup>T\u7ec6\u80de\u6570\u91cf\u8981\u8d85\u8fc7CD8<sup>+<\/sup>T\u7ec6\u80de\uff0c\u53ef\u89c1\u7c92\u7ec6\u80de\u6d78\u6da6\uff0c\u800c\u5728MS\u4e2d\u4e3b\u8981\u7531\u6d3b\u5316\u7684\u5de8\u566c\u7ec6\u80de\u3001\u5c0f\u80f6\u8d28\u7ec6\u80de\u548cCD8<sup>+<\/sup>T\u6dcb\u5df4\u7ec6\u80de\u7ec4\u6210\uff0cCD4<sup>+<\/sup>T\u7ec6\u80de\u3001B\u6dcb\u5df4\u7ec6\u80de\u548c\u6d46\u7ec6\u80de\u7684\u6570\u91cf\u8f83\u5c11\uff0c\u7c92\u7ec6\u80de\u6d78\u6da6\u4e0d\u5e38\u89c1<sup>\uff3b<\/sup><sup>24\uff3d<\/sup>\u3002<\/p>\n<p>\u76f8\u5173\u7814\u7a76\u7ed3\u679c\u8868\u660e\uff0c\u5728NMO\u5c0f\u9f20\u6a21\u578b\u4e2d\uff0c\u5916\u5468\u8840\u6db2\u5faa\u73af\u4e2d\u7684\u4e2d\u6027\u7c92\u7ec6\u80de\u663e\u8457\u5347\u9ad8\uff0c\u5728\u75c5\u53d8\u65e9\u671f\u8fdb\u5165CNS\u540e\u53ef\u52a0\u5267\u8865\u4f53\u6fc0\u6d3b\u5f15\u8d77\u7684\u635f\u4f24\uff0c\u4e2d\u6027\u7c92\u7ec6\u80de\u4e3b\u8981\u4f9d\u8d56\u5f39\u6027\u86cb\u767d\u9176\u4ea7\u751fCNS\u635f\u4f24\uff0c\u5b9e\u9a8c\u7ed3\u679c\u4e5f\u8868\u660e\u9009\u62e9\u6027\u5f39\u6027\u86cb\u767d\u6291\u5236\u5242\uff08\u897f\u7ef4\u6765\u53f8\uff09\u5bf9NMO\u6025\u6027\u671f\u6cbb\u7597\u6709\u76ca<sup>\uff3b<\/sup><sup>40\uff3d<\/sup>\u3002<\/p>\n<p>\u55dc\u9178\u6027\u7c92\u7ec6\u80de\u5728NMO\u8131\u9ad3\u9798\u75c5\u53d8\u4e2d\u542b\u91cf\u4e30\u5bcc\uff0c\u7814\u7a76\u53d1\u73b0\u5176\u53ef\u80fd\u662f\u901a\u8fc7\u4e0e\u7ec6\u80de\u8868\u9762\u7ed3\u5408\u7684\u6297\u4f53\u5171\u540c\u4f5c\u7528\u4ea7\u751f\u6297\u4f53\u4f9d\u8d56\u6027\u7ec6\u80de\u4ecb\u5bfc\u7684\u7ec6\u80de\u6bd2\u6027\uff0c\u6216\u5728\u8865\u4f53\u7684\u5e2e\u52a9\u4e0b\uff0c\u901a\u8fc7\u8865\u4f53\u4f9d\u8d56\u6027\u7ec6\u80de\u4ecb\u5bfc\u7684\u7ec6\u80de\u6bd2\u6027\u6740\u4f24\u7ec6\u80de\uff0c\u800c\u6297\u7ec4\u80fa\u836f\u897f\u66ff\u5229\u55ea\u5bf9\u9020\u6a21\u5c0f\u9f20\u7684\u75c5\u53d8\u6709\u7f13\u89e3\u4f5c\u7528<sup>\uff3b<\/sup><sup>41\uff3d<\/sup>\u3002<\/p>\n<p>\u8fd1\u5e74\u6765\uff0c\u5de8\u566c\u7ec6\u80de\/\u5c0f\u80f6\u8d28\u7ec6\u80de\u5728NMO\u53d1\u75c5\u673a\u5236\u4e2d\u7684\u4f5c\u7528\u53d7\u5230\u91cd\u89c6\u3002\u7814\u7a76\u8ba4\u4e3a\u5de8\u566c\u7ec6\u80de\/\u5c0f\u80f6\u8d28\u7ec6\u80de\u5728\u7ec6\u80de\u6eb6\u89e3\u524d\u671f\u4e0e\u661f\u5f62\u80f6\u8d28\u7ec6\u80de\u7684\u76f8\u4e92\u4f5c\u7528\u53ef\u80fd\u662f\u9a71\u52a8NMO\u75c5\u53d8\u7684\u5173\u952e\uff0c\u6b64\u8fc7\u7a0b\u6d89\u53ca\u65e9\u671f\u6fc0\u6d3b\u7684\u8865\u4f53\u6210\u5206\u4e0e\u5c0f\u80f6\u8d28\u7ec6\u80deC3a\u53d7\u4f53\u4fe1\u53f7\uff0cAQP4-IgG\u6fc0\u6d3b\u7684\u661f\u578b\u80f6\u8d28\u7ec6\u80de\u53ef\u80fd\u901a\u8fc7C3\u88c2\u89e3\u4ea7\u7269C3a\u4fc3\u8fdb\u5de8\u566c\u7ec6\u80de\/\u5c0f\u80f6\u8d28\u7ec6\u80de\u7684\u6d3b\u5316\uff0c\u6fc0\u6d3b\u7684\u5de8\u566c\u7ec6\u80de\/\u5c0f\u80f6\u8d28\u7ec6\u80de\u4ea7\u751f\u7684C1q\u53ef\u4ee5\u542f\u52a8\u7ecf\u5178\u8865\u4f53\u7ea7\u8054\u53cd\u5e94\uff0c\u4e5f\u53ef\u80fd\u5355\u72ec\u5f15\u8d77\u8f74\u7a81\u635f\u4f24\uff0c\u6545\u5de8\u566c\u7ec6\u80de\/\u5c0f\u80f6\u8d28\u7ec6\u80de\u53ef\u80fd\u662f\u6cbb\u7597NMO\u7684\u65b0\u9776\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amino acid transporter 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