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\u86cb\u767d\u8cea\u932f\u8aa4\u6298\u758a\u5f15\u767c\u5c0f\u8166\u840e\u7e2e\u75c7\u4e09\u578b<\/strong>\u00a0<\/strong><\/p>\r\n

\u86cb\u767d\u8cea\u5728\u4eba\u9ad4\u7d30\u80de\u4e2d\u64d4\u7576\u91cd\u8981\u89d2\u8272\uff0c\u5305\u62ec\u767c\u5c55\u53ca\u7dad\u6301\u795e\u7d93\u5143\u7d30\u80de\u7684\u529f\u80fd\u3002\u4e0d\u540c\u7814\u7a76\u8b49\u5be6\uff0c\u932f\u8aa4\u647a\u758a\u548c\u805a\u96c6\u7684\u86cb\u767d\u8cea\u8207\u591a\u7a2e\u4eba\u985e\u75be\u75c5\u606f\u606f\u76f8\u95dc\u3002\u86cb\u767d\u8cea\u5728\u7d30\u80de\u4e2d\u5fc5\u9808\u7d93\u904e\u6b63\u78ba\u647a\u758a\u9054\u5230\u7684\u529f\u80fd\u6027\u7d50\u69cb\u624d\u80fd\u6b63\u5e38\u904b\u4f5c\uff0c\u4e00\u65e6\u767c\u751f\u932f\u8aa4\u647a\u758a\uff0c\u5373\u4f7f\u6975\u70ba\u8f15\u5fae\uff0c\u4e5f\u53ef\u80fd\u4ee4\u7d30\u80de\u529f\u80fd\u51fa\u73fe\u7570\u5e38\uff0c\u5f62\u6210\u5e36\u6bd2\u6027\u53ca\u7121\u6cd5\u6eb6\u89e3\u7684\u86cb\u767d\u805a\u5408\u7269\uff0c\u5f9e\u800c\u5f15\u767c\u75be\u75c5\u3002\u5728\u5c0f\u8166\u840e\u7e2e\u75c7\u4e09\u578b\u8207\u5176\u4ed6\u591a\u805a\u8c37\u6c28\u9170\u80fa\uff08polyQ\uff09\u7b49\u795e\u7d93\u7cfb\u7d71\u75be\u75c5\u4e2d\uff0c\u932f\u8aa4\u647a\u758a\u7684\u86cb\u767d\u53ca\u805a\u5408\u7269\u6703\u805a\u96c6\u65bc\u9000\u5316\u7684\u795e\u7d93\u5143\u4e2d\uff0c\u5f71\u97ff\u5176\u4ed6\u86cb\u767d\u7684\u6b63\u5e38\u529f\u80fd\u3002<\/p>\r\n

Prpf19<\/strong>\u6709\u52a9\u964d\u89e3\u5e36\u6bd2\u6027\u7684\u5c0f\u8166\u840e\u7e2e\u75c7\u4e09\u578b\u86cb\u767d<\/strong>\u00a0<\/strong><\/p>\r\n

\u9673\u6d69\u7136\u6559\u6388\u3001\u535a\u58eb\u5f8c\u7814\u7a76\u54e1\u9673\u54f2\u51e1\u535a\u58eb\u53ca\u5176\u7814\u7a76\u5718\u968a\u767c\u73fe\u5c0f\u8166\u840e\u7e2e\u75c7\u4e09\u578b\u75be\u75c5\u86cb\u767d\u7684\u6c34\u5e73\u8207\u7d30\u80de\u6838\u5167\u5b9a\u4f4d\u7684Prpf19\u86cb\u767d\u6709\u5bc6\u5207\u7684\u95dc\u4fc2\uff0c\u7576Prpf19\u529f\u80fd\u589e\u5f37\u6642\uff0c\u5c0f\u8166\u840e\u7e2e\u75c7\u4e09\u578b\u75be\u75c5\u86cb\u767d\u6703\u901a\u904e\u300c\u6cdb\u7d20-\u86cb\u767d\u9176\u9ad4\u7cfb\u7d71\u300d\u53d7\u5230\u964d\u89e3\uff0c\u6e1b\u4f4e\u5c0f\u8166\u840e\u7e2e\u75c7\u4e09\u578b\u7684\u7d30\u80de\u6bd2\u6027\uff0c\u800c\u795e\u7d93\u9000\u5316\u60c5\u6cc1\u5728\u52d5\u7269\u6a21\u578b\u4e2d\u4e5f\u5f97\u5230\u6539\u5584\u3002<\/p>\r\n

Prpf19<\/strong>\u8207<\/strong>Exoc7<\/strong>\u9593\u7684\u62ee\u6297\u4f5c\u7528\u8abf\u63a7\u5c0f\u8166\u840e\u7e2e\u75c7\u4e09\u578b\u7684\u795e\u7d93\u9000\u5316<\/strong><\/p>\r\n

Exoc7\u86cb\u767d\u8ca0\u8cac\u63a7\u5236\u7d30\u80de\u5167\u86cb\u767d\u8cea\u7684\u904b\u8f38\uff0c\u8207Prpf19\u86cb\u767d\u5728\u57f7\u884c\u591a\u7a2e\u7d30\u80de\u529f\u80fd\u6642\u8d77\u4ea4\u4e92\u4f5c\u7528\uff0c\u800cExoc7\u86cb\u767d\u7684\u6372\u66f2\u87ba\u65cb\u578b\u86cb\u767d\u7d50\u69cb\u662f\u6291\u5236Prpf19\u529f\u80fd\u7684\u95dc\u9375\u90e8\u5206\u3002\u7814\u7a76\u5718\u968a\u66f4\u767c\u73fe\uff0cExoc7\u6703\u904a\u8d70\u5230\u7d30\u80de\u6838\uff0c\u6e1b\u4f4ePrpf19\u5c0d\u75be\u75c5\u86cb\u767d\u7684\u5206\u89e3\uff0c\u4e26\u5e72\u64fepre-mRNA\u86cb\u767d\u8cea\u526a\u63a5\uff0c\u5c0e\u81f4Prpf19\u5931\u53bb\u5c0d\u5c0f\u8166\u840e\u7e2e\u75c7\u4e09\u578b\u6a21\u578b\u795e\u7d93\u9000\u5316\u7684\u4fdd\u8b77\u529f\u80fd\u3002<\/p>\r\n

\u9673\u6559\u6388\u8868\u793a\uff1a\u300c\u662f\u6b21\u7814\u7a76\u63ed\u793a\u4e86Prpf19\u8207Exoc7\u4e4b\u9593\u8907\u96dc\u7684\u86cb\u767d\u62ee\u6297\u4f5c\u7528\uff0c\u9032\u4e00\u6b65\u7814\u7a76\u86cb\u767d\u8cea\u7db2\u7d61\u63a7\u5236\u86cb\u767d\u805a\u96c6\u7684\u4f5c\u7528\u6a5f\u5236\uff0c\u5c07\u6709\u52a9\u6211\u5011\u958b\u767c\u91dd\u5c0dPrpf19\u7684\u6f5b\u5728\u5206\u5b50\u6216\u6fc0\u6d3b\u5291\uff0c\u5e0c\u671b\u80fd\u70ba\u5c0f\u8166\u840e\u7e2e\u75c7\u4e09\u578b\u53ca\u5176\u4ed6\u795e\u7d93\u9000\u884c\u6027\u75be\u75c5\u63d0\u4f9b\u65b0\u7684\u6cbb\u7642\u65b9\u6848\u30022\u670828\u65e5\u70ba2021\u4e16\u754c\u7f55\u898b\u75c5\u65e5(https:\/\/www.rarediseaseday.org\/<\/a>)\uff0c\u5c0f\u8166\u840e\u7e2e\u75c7\u4e09\u578b\u5c6c\u7f55\u898b\u75be\u75c5\u7684\u4e00\u7a2e\uff0c\u76fc\u671b\u6211\u5011\u7684\u7814\u7a76\u6210\u679c\u80fd\u63a8\u52d5\u7f55\u898b\u75be\u75c5\u7684\u8f49\u5316\u91ab\u5b78\u7814\u7a76\u3002\u300d<\/p>\r\n

\u9019\u9805\u7814\u7a76\u7372\u4e2d\u5927\u91ab\u5b78\u9662\u8521\u6c38\u696d\u8166\u795e\u7d93\u79d1\u5b78\u4e2d\u5fc3\u3001\u9999\u6e2f\u7814\u7a76\u8cc7\u52a9\u5c40\u512a\u914d\u7814\u7a76\u91d1\u53ca\u5353\u8d8a\u5b78\u79d1\u9818\u57df\u8a08\u5283\u7684\u8cc7\u52a9\u3002 \u8ad6\u6587\u5168\u6587\u8acb\u53c3\u95b1\ufe55https:\/\/www.nature.com\/articles\/s41419-021-03444-x<\/a>.\u00a0<\/p>\r\n

\u9673\u6d69\u7136\u6559\u6388\u7c21\u6b77<\/strong><\/p>\r\n

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\u9673\u54f2\u51e1\u535a\u58eb\u7c21\u6b77<\/strong><\/p>\r\n

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