{"id":10695,"date":"2025-07-15T03:32:50","date_gmt":"2025-07-14T19:32:50","guid":{"rendered":"https:\/\/fanyiming.life\/?p=10695"},"modified":"2025-12-21T01:03:24","modified_gmt":"2025-12-20T17:03:24","slug":"rtcb-ddr%e8%bd%b4%ef%bc%9a%e6%8f%ad%e7%a4%barna%e8%bf%9e%e6%8e%a5%e4%b8%8e%e5%9f%ba%e5%9b%a0%e7%bb%84%e7%a8%b3%e5%ae%9a%e6%80%a7%e4%b9%8b%e9%97%b4%e7%9a%84%e7%9b%b8%e4%ba%92%e4%bd%9c%e7%94%a8","status":"publish","type":"post","link":"https:\/\/fanyiming.life\/?p=10695","title":{"rendered":"RTCB-DDR\u8f74\uff1a\u63ed\u793aRNA\u8fde\u63a5\u4e0e\u57fa\u56e0\u7ec4\u7a33\u5b9a\u6027\u4e4b\u95f4\u7684\u76f8\u4e92\u4f5c\u7528"},"content":{"rendered":"\n

\u6267\u884c\u6458\u8981<\/h3>\n\n\n\n

\u672c\u62a5\u544a\u65e8\u5728\u8be6\u5c3d\u9610\u8ff0RNA 3′-\u672b\u7aef\u78f7\u9178\u73af\u5316\u9176\uff08RTCB\uff09\u4e0e\u7ec6\u80deDNA\u635f\u4f24\u4fee\u590d\uff08DDR\uff09\u673a\u5236\u4e4b\u95f4\u7684\u590d\u6742\u5173\u8054\u3002RTCB\u4f5c\u4e3a\u4e00\u79cd\u5173\u952e\u7684RNA\u8fde\u63a5\u9176\uff0c\u5176\u5728tRNA\u6210\u719f\u548c\u672a\u6298\u53e0\u86cb\u767d\u53cd\u5e94\uff08UPR\uff09\u4e2d\u7684\u6838\u5fc3\u4f5c\u7528\u5df2\u5f97\u5230\u5145\u5206\u8bc1\u5b9e\u3002\u7136\u800c\uff0c\u8d8a\u6765\u8d8a\u591a\u7684\u8bc1\u636e\u8868\u660e\uff0cRTCB\u7684\u529f\u80fd\u8fdc\u4e0d\u6b62\u4e8eRNA\u4ee3\u8c22\uff0c\u5b83\u4e0e\u7ef4\u6301\u57fa\u56e0\u7ec4\u7a33\u5b9a\u6027\u7684DDR\u7f51\u7edc\u5b58\u5728\u7740\u6df1\u523b\u800c\u591a\u7ef4\u5ea6\u7684\u8054\u7cfb\u3002\u672c\u62a5\u544a\u5c06\u7cfb\u7edf\u6027\u5730\u5256\u6790\u8fd9\u4e9b\u8054\u7cfb\uff0c\u5305\u62ec\uff1a\uff081\uff09RTCB\u5bb6\u65cf\u6210\u5458\u5728\u539f\u6838\u751f\u7269\u4e2d\u8868\u73b0\u51fa\u7684\u76f4\u63a5\u4fee\u9970DNA\u5e95\u7269\u7684\u9176\u6d3b\u6027\uff1b\uff082\uff09\u5728\u540e\u751f\u52a8\u7269\u4e2d\uff0cRTCB\u901a\u8fc7\u5176\u6240\u5728\u7684tRNA\u8fde\u63a5\u9176\u590d\u5408\u7269\uff08tRNA-LC\uff09\u4e2d\u7684DDX1\u4e9a\u57fa\u4e0eDDR\u673a\u5236\u95f4\u63a5\u5173\u8054\uff1b\uff083\uff09\u5728\u57fa\u56e0\u6bd2\u6027\u5e94\u6fc0\u4e0b\uff0cRTCB\u901a\u8def\u7684\u8868\u8fbe\u88ab\u4e0a\u8c03\uff0c\u4f5c\u4e3a\u4e00\u79cd\u652f\u6301\u6027\u4fee\u590d\u53cd\u5e94\uff1b\uff084\uff09\u5728\u54fa\u4e73\u52a8\u7269\u6a21\u578b\u4e2d\uff0cRTCB\u901a\u8fc7\u8c03\u63a7DDR\u76f8\u5173\u57fa\u56e0\u7684\u526a\u63a5\uff0c\u5bf9\u6574\u4e2a\u4fee\u590d\u7f51\u7edc\u884c\u4f7f\u4e0a\u6e38\u8c03\u63a7\u529f\u80fd\u3002\u7efc\u5408\u8fd9\u4e9b\u8bc1\u636e\uff0c\u672c\u62a5\u544a\u65e8\u5728\u6784\u5efa\u4e00\u4e2a\u7edf\u4e00\u6a21\u578b\uff0c\u63ed\u793aRTCB\u4f5c\u4e3a\u4e00\u4e2a\u8fde\u63a5RNA\u52a0\u5de5\u3001\u86cb\u767d\u8d28\u5408\u6210\u548c\u57fa\u56e0\u7ec4\u7ef4\u62a4\u7684\u5173\u952e\u8282\u70b9\uff0c\u5728\u7ec6\u80de\u5e94\u5bf9\u9057\u4f20\u7269\u8d28\u635f\u4f24\u7684\u7efc\u5408\u7b56\u7565\u4e2d\u626e\u6f14\u7740\u81f3\u5173\u91cd\u8981\u7684\u89d2\u8272\u3002<\/p>\n\n\n\n

I. RTCB\u7684\u7ecf\u5178\u89d2\u8272\uff1aRNA\u6210\u719f\u4e0e\u7ec6\u80de\u7a33\u6001\u7684\u4e3b\u8c03\u8282\u5668<\/h2>\n\n\n\n

\u5728\u63a2\u7a76RTCB\u4e0eDNA\u635f\u4f24\u4fee\u590d\u4e4b\u95f4\u7684\u65b0\u5174\u5173\u8054\u4e4b\u524d\uff0c\u5fc5\u987b\u9996\u5148\u660e\u786e\u5176\u5728\u7ec6\u80de\u5185\u5df2\u77e5\u7684\u3001\u57fa\u7840\u6027\u7684\u529f\u80fd\u3002RTCB\u4f5c\u4e3a\u4e00\u79cd\u9ad8\u5ea6\u4fdd\u5b88\u7684\u9176\uff0c\u662f\u7ef4\u6301\u86cb\u767d\u8d28\u5408\u6210\u548c\u7ec6\u80de\u5e94\u6fc0\u53cd\u5e94\u6b63\u5e38\u8fd0\u4f5c\u7684\u57fa\u77f3\u3002<\/p>\n\n\n\n

A. RTCB\u4f5c\u4e3atRNA\u526a\u63a5\u7684\u5173\u952e\u8fde\u63a5\u9176<\/h3>\n\n\n\n

RTCB\u6700\u6838\u5fc3\u7684\u529f\u80fd\u4e4b\u4e00\u662f\u50ac\u5316\u542b\u5185\u542b\u5b50tRNA\u524d\u4f53\u7684\u6210\u719f\u3002\u5728\u53e4\u83cc\u548c\u540e\u751f\u52a8\u7269\u4e2d\uff0c\u4e00\u90e8\u5206tRNA\u57fa\u56e0\u5305\u542b\u5185\u542b\u5b50\uff0c\u8fd9\u4e9b\u5185\u542b\u5b50\u5fc5\u987b\u5728\u8f6c\u5f55\u540e\u88ab\u7cbe\u786e\u5207\u9664\uff0c\u968f\u540e\u7531RTCB\u5c06\u4e24\u4e2atRNA\u5916\u663e\u5b50\u534a\u4f53\u91cd\u65b0\u8fde\u63a5\u8d77\u6765 1<\/sup>\u3002\u8fd9\u4e00\u8fc7\u7a0b\u5bf9\u4e8e\u4ea7\u751f\u529f\u80fd\u6027tRNA\u81f3\u5173\u91cd\u8981\u3002\u4f8b\u5982\uff0c\u5728\u4eba\u7c7b\u57fa\u56e0\u7ec4\u4e2d\uff0c416\u4e2atRNA\u57fa\u56e0\u4e2d\u670928\u4e2a\u542b\u6709\u5185\u542b\u5b50\uff0c\u5176\u4e2d\u5305\u62ec\u6240\u670913\u4e2a\u7f16\u7801\u916a\u6c28\u9178tRNA\u7684\u57fa\u56e0\uff0c\u8fd9\u4f7f\u5f97RTCB\u7684\u8fde\u63a5\u6d3b\u6027\u6210\u4e3a\u901a\u7528\u86cb\u767d\u8d28\u7ffb\u8bd1\u4e0d\u53ef\u6216\u7f3a\u7684\u4e00\u73af 4<\/sup>\u3002<\/p>\n\n\n\n

\u5728\u7ebf\u866b\uff08Caenorhabditis elegans<\/em>\uff09\u4e2d\u7684\u9057\u4f20\u5b66\u7814\u7a76\u4e3aRTCB\u7684\u4f53\u5185\u529f\u80fd\u63d0\u4f9b\u4e86\u51b3\u5b9a\u6027\u8bc1\u636e\u3002RtcB<\/em>\u57fa\u56e0\u7684\u6572\u9664\u5bfc\u81f4\u672a\u8fde\u63a5\u7684tRNA\u534a\u4f53\u5927\u91cf\u79ef\u7d2f\uff0c\u800c\u6210\u719f\u7684\u3001\u526a\u63a5\u540e\u7684tRNA\u6c34\u5e73\u5219\u663e\u8457\u4e0b\u964d 2<\/sup>\u3002\u8fd9\u4e9b<\/p>\n\n\n\n

RtcB<\/em>\u7a81\u53d8\u4f53\u8868\u73b0\u51fa\u751f\u957f\u8fdf\u7f13\u548c\u5bff\u547d\u7f29\u77ed\u7684\u7f3a\u9677\uff0c\u800c\u8fd9\u4e9b\u7f3a\u9677\u53ef\u4ee5\u901a\u8fc7\u5728\u7a81\u53d8\u4f53\u4e2d\u8868\u8fbe\u9884\u5148\u526a\u63a5\u597d\u7684tRNA\u6765\u90e8\u5206\u633d\u6551 2<\/sup>\u3002\u8fd9\u4e00\u7ed3\u679c\u6709\u529b\u5730\u8bc1\u660e\u4e86RTCB\u7684tRNA\u8fde\u63a5\u6d3b\u6027\u5bf9\u4e8e\u751f\u7269\u4f53\u7684\u6b63\u5e38\u53d1\u80b2\u548c\u751f\u5b58\u81f3\u5173\u91cd\u8981\uff0c\u5e76\u5c06\u5176\u5b9a\u4f4d\u4e3a\u86cb\u767d\u8d28\u5408\u6210\u548c\u7ec6\u80de\u7a33\u6001\u7684\u6838\u5fc3\u8c03\u63a7\u56e0\u5b50\u3002<\/p>\n\n\n\n

B. RTCB\u5728\u672a\u6298\u53e0\u86cb\u767d\u53cd\u5e94\uff08UPR\uff09\u4e2d\u7684\u5173\u952e\u529f\u80fd<\/h3>\n\n\n\n

\u9664\u4e86\u5728tRNA\u6210\u719f\u4e2d\u7684\u4f5c\u7528\uff0cRTCB\u5728\u7ec6\u80de\u5e94\u6fc0\u53cd\u5e94\u4e2d\u4e5f\u626e\u6f14\u7740\u4e0d\u53ef\u66ff\u4ee3\u7684\u89d2\u8272\u3002\u5728\u540e\u751f\u52a8\u7269\u4e2d\uff0cRTCB\u662f\u672a\u6298\u53e0\u86cb\u767d\u53cd\u5e94\uff08UPR\uff09IRE1\u5206\u652f\u4e2d\u552f\u4e00\u7684mRNA\u8fde\u63a5\u9176 2<\/sup>\u3002UPR\u662f\u7ec6\u80de\u5e94\u5bf9\u5185\u8d28\u7f51\u4e2d\u672a\u6298\u53e0\u6216\u9519\u8bef\u6298\u53e0\u86cb\u767d\u79ef\u7d2f\u7684\u4e00\u79cd\u9002\u5e94\u6027\u673a\u5236\u3002\u5728\u6b64\u8fc7\u7a0b\u4e2d\uff0cIRE1\u6838\u9178\u5185\u5207\u9176\u4f1a\u5207\u9664XBP1\u8f6c\u5f55\u56e0\u5b50\u7684mRNA\u4e2d\u7684\u4e00\u4e2a\u77ed\u5185\u542b\u5b50\u3002\u968f\u540e\uff0cRTCB\u8d1f\u8d23\u5c06\u4e24\u4e2amRNA\u5916\u663e\u5b50\u7247\u6bb5\u8fde\u63a5\u8d77\u6765\uff0c\u4ea7\u751f\u4e00\u4e2a\u526a\u63a5\u540e\u7684\u3001\u5177\u6709\u6d3b\u6027\u7684<\/p>\n\n\n\n

XBP1s<\/em> mRNA 5<\/sup>\u3002<\/p>\n\n\n\n

\u5728RtcB<\/em>\u7a81\u53d8\u52a8\u7269\u4e2d\uff0cUPR\u901a\u8def\u5b58\u5728\u4e25\u91cd\u7f3a\u9677\u3002\u5f53\u7528\u8863\u9709\u7d20\u7b49\u836f\u7269\u8bf1\u5bfc\u86cb\u767d\u8d28\u5e94\u6fc0\u65f6\uff0c\u7a81\u53d8\u4f53\u7ec6\u80de\u5185\u4f1a\u79ef\u7d2f\u5927\u91cf\u672a\u8fde\u63a5\u7684xbp-1<\/em> mRNA\u7247\u6bb5\uff0c\u800c\u65e0\u6cd5\u4ea7\u751f\u6709\u6d3b\u6027\u7684xbp-1s<\/em>\u3002\u8fd9\u5bfc\u81f4UPR\u4e0b\u6e38\u9776\u57fa\u56e0\u65e0\u6cd5\u88ab\u6fc0\u6d3b\uff0c\u4f7f\u52a8\u7269\u5728\u5e94\u6fc0\u6761\u4ef6\u4e0b\u7684\u751f\u5b58\u80fd\u529b\u548c\u5bff\u547d\u663e\u8457\u964d\u4f4e 2<\/sup>\u3002\u56e0\u6b64\uff0cRTCB\u662f\u8fde\u63a5\u5185\u8d28\u7f51\u7a33\u6001\u4e0e\u57fa\u56e0\u8868\u8fbe\u8c03\u63a7\u7684\u5173\u952e\u5206\u5b50\uff0c\u5176\u529f\u80fd\u969c\u788d\u4f1a\u4e25\u91cd\u635f\u5bb3\u7ec6\u80de\u5e94\u5bf9\u86cb\u767d\u8d28\u6bd2\u6027\u5e94\u6fc0\u7684\u80fd\u529b\u3002<\/p>\n\n\n\n

C. RTCB\u8fde\u63a5\u53cd\u5e94\u7684\u72ec\u7279\u751f\u5316\u673a\u5236<\/h3>\n\n\n\n

RTCB\u7684\u9176\u5b66\u673a\u5236\u4e0e\u7ecf\u5178\u7684\u6838\u9178\u8fde\u63a5\u9176\u622a\u7136\u4e0d\u540c\uff0c\u8fd9\u51f8\u663e\u4e86\u5176\u5728\u751f\u7269\u5b66\u4e2d\u7684\u72ec\u7279\u6027\u3002\u5927\u591a\u6570\u8fde\u63a5\u9176\uff0c\u5982DNA\u8fde\u63a5\u9176\u548cT4 RNA\u8fde\u63a5\u9176\uff0c\u662fATP\u4f9d\u8d56\u6027\u7684\uff0c\u5e76\u901a\u8fc7\u6fc0\u6d3b\u5e95\u7269\u76845′-\u78f7\u9178\u672b\u7aef\u6765\u50ac\u5316\u8fde\u63a5\u53cd\u5e94 6<\/sup>\u3002\u76f8\u6bd4\u4e4b\u4e0b\uff0cRTCB\u662f\u4e00\u79cdGTP\u4f9d\u8d56\u6027\u7684\u8fde\u63a5\u9176\uff0c\u5b83\u4f5c\u7528\u4e8e\u5177\u67093′-\u78f7\u9178\uff08\u62162′,3′-\u73af\u78f7\u9178\uff09\u548c5′-\u7f9f\u57fa\u672b\u7aef\u7684\u5e95\u7269 7<\/sup>\u3002<\/p>\n\n\n\n

RTCB\u7684\u50ac\u5316\u53cd\u5e94\u4e25\u683c\u4f9d\u8d56\u4e8eGTP\u4f5c\u4e3a\u80fd\u91cf\u6765\u6e90\u548c\u4e8c\u4ef7\u9530\u79bb\u5b50\uff08Mn2+\uff09\u4f5c\u4e3a\u8f85\u56e0\u5b50\uff0c\u5728\u6ca1\u6709GTP\u6216\u4f7f\u7528\u5176\u4ed6\u4e8c\u4ef7\u9633\u79bb\u5b50\uff08\u5982Mg2+\uff09\u7684\u60c5\u51b5\u4e0b\u5219\u5b8c\u5168\u6ca1\u6709\u6d3b\u6027 6<\/sup>\u3002\u5176\u72ec\u7279\u7684\u8fde\u63a5\u8fc7\u7a0b\u9075\u5faa\u4e00\u4e2a\u4e09\u6b65\u53cd\u5e94\u901a\u8def 6<\/sup>\uff1a<\/p>\n\n\n\n

    \n
  1. \u9176\u7684\u9e1f\u82f7\u9170\u5316\uff08Guanylylation\uff09<\/strong>\uff1aRTCB\u7684\u6d3b\u6027\u4f4d\u70b9\u7ec4\u6c28\u9178\uff08\u4f8b\u5982\uff0c\u5728Pyrococcus horikoshii<\/em>\u4e2d\u662fHis404\uff0c\u5728E. coli<\/em>\u4e2d\u662fHis337\uff09\u5bf9GTP\u7684$\\alpha$-\u78f7\u9178\u57fa\u56e2\u8fdb\u884c\u4eb2\u6838\u653b\u51fb\uff0c\u5f62\u6210\u4e00\u4e2a\u5171\u4ef7\u7684RtcB-(\u7ec4\u6c28\u9170-N\u03b5)-GMP\u4e2d\u95f4\u4f53\uff0c\u5e76\u91ca\u653e\u7126\u78f7\u9178 3<\/sup>\u3002<\/li>\n\n\n\n
  2. \u9e1f\u82f7\u9170\u57fa\u8f6c\u79fb<\/strong>\uff1aGMP\u90e8\u5206\u4ece\u9176\u8f6c\u79fb\u5230RNA\u5e95\u7269\u76843′-\u78f7\u9178\u672b\u7aef\uff0c\u5f62\u6210\u4e00\u4e2a\u9ad8\u80fd\u7684RNA-(3′)pp(5′)G\u6d3b\u5316\u4e2d\u95f4\u4f53 7<\/sup>\u3002<\/li>\n\n\n\n
  3. \u78f7\u9178\u4e8c\u916f\u952e\u5f62\u6210<\/strong>\uff1a\u7b2c\u4e8c\u4e2aRNA\u94fe\u76845′-\u7f9f\u57fa\u4eb2\u6838\u653b\u51fb\u88ab\u6d3b\u5316\u76843’\u672b\u7aef\uff0c\u5f62\u6210\u6700\u7ec8\u76843′-5’\u78f7\u9178\u4e8c\u916f\u952e\uff0c\u5e76\u91ca\u653eGMP 7<\/sup>\u3002<\/li>\n<\/ol>\n\n\n\n

    \u5bf9Pyrococcus horikoshii<\/em> RtcB\u7684\u7ed3\u6784\u751f\u7269\u5b66\u7814\u7a76\u63ed\u793a\uff0c\u5176\u6d3b\u6027\u4e2d\u5fc3\u542b\u6709\u4e00\u4e2a\u53cc\u6838$Mn^{2+}$\u4e2d\u5fc3\uff0c\u8fd9\u5bf9\u4e8e\u7cbe\u786e\u534f\u8c03GTP\u548c\u5e95\u7269\u78f7\u9178\u57fa\u56e2\uff0c\u4ece\u800c\u6709\u6548\u50ac\u5316\u4e0a\u8ff0\u4e00\u7cfb\u5217\u6838\u82f7\u9170\u57fa\u8f6c\u79fb\u53cd\u5e94\u81f3\u5173\u91cd\u8981 6<\/sup>\u3002<\/p>\n\n\n\n

    RTCB\u72ec\u7279\u7684GTP\/$Mn^{2+}$\u4f9d\u8d56\u6027\u548c3’\u672b\u7aef\u6fc0\u6d3b\u673a\u5236\uff0c\u4e0d\u4ec5\u4ec5\u662f\u4e00\u4e2a\u751f\u5316\u7ec6\u8282\u3002\u5b83\u6807\u5fd7\u7740\u4e00\u6761\u4e0e\u5e9e\u5927\u7684ATP\/$Mg^{2+}$\u4f9d\u8d56\u6027\u8fde\u63a5\u9176\u5bb6\u65cf\uff08\u5305\u62ec\u51e0\u4e4e\u6240\u6709DNA\u8fde\u63a5\u9176\uff09\u5b8c\u5168\u4e0d\u540c\u7684\u8fdb\u5316\u9014\u5f84\u548c\u8c03\u63a7\u903b\u8f91\u3002\u8fd9\u610f\u5473\u7740\uff0c\u8c03\u63a7RTCB\u6d3b\u6027\u7684\u7ec6\u80de\u4fe1\u53f7\u53ef\u80fd\u4e0e\u8c03\u63a7\u7ec6\u80de\u5185\u7edd\u5927\u591a\u6570\u8fde\u63a5\u53cd\u5e94\u7684\u4fe1\u53f7\u622a\u7136\u4e0d\u540c\u3002\u7ec6\u80de\u5185\u7684GTP\u6c34\u5e73\u548c\u9530\u79bb\u5b50\u6d53\u5ea6\uff0c\u4e24\u8005\u90fd\u53ef\u80fd\u5728\u4e0d\u540c\u7684\u4ee3\u8c22\u548c\u5e94\u6fc0\u6761\u4ef6\u4e0b\u53d1\u751f\u6ce2\u52a8\uff0c\u4ece\u800c\u80fd\u591f\u4ee5\u4e00\u79cdATP\u4f9d\u8d56\u6027\u8fc7\u7a0b\u6240\u4e0d\u5177\u5907\u7684\u65b9\u5f0f\u76f4\u63a5\u8c03\u8282RTCB\u7684\u6d3b\u6027\u3002\u8fd9\u79cd\u72ec\u7279\u7684\u751f\u5316\u7279\u6027\u4f7fRTCB\u6210\u4e3a\u4e00\u4e2a\u9ad8\u5ea6\u7279\u5f02\u7684\u8c03\u63a7\u8282\u70b9\uff0c\u4e0d\u4ec5\u63a7\u5236\u7740tRNA\u6210\u719f\u548cUPR\uff0c\u4e5f\u5f15\u51fa\u4e86\u4e00\u4e2a\u91cd\u8981\u95ee\u9898\uff1a\u5982\u679cRTCB\u53c2\u4e0eDNA\u4fee\u590d\uff0c\u5b83\u5f88\u53ef\u80fd\u662f\u5728\u4e00\u4e2a\u7279\u6b8a\u7684\u80cc\u666f\u4e0b\uff0c\u5904\u7406\u7ecf\u5178DNA\u8fde\u63a5\u9176\u65e0\u6cd5\u8bc6\u522b\u7684\u975e\u5178\u578bDNA\u672b\u7aef\uff0c\u6216\u662f\u88ab\u901a\u8fc7GTP\/GDP\u6bd4\u7387\u4f20\u9012\u7684\u7279\u5b9a\u5e94\u6fc0\u4fe1\u53f7\u6240\u8c03\u63a7\u3002<\/p>\n\n\n\n

    II. \u7ec6\u80deDNA\u635f\u4f24\u4fee\u590d\uff1a\u57fa\u56e0\u7ec4\u5b8c\u6574\u6027\u7684\u591a\u5c42\u6b21\u9632\u5fa1\u4f53\u7cfb<\/h2>\n\n\n\n

    \u4e3a\u4e86\u7406\u89e3RTCB\u53ef\u80fd\u5728DNA\u635f\u4f24\u4fee\u590d\uff08DDR\uff09\u4e2d\u626e\u6f14\u7684\u89d2\u8272\uff0c\u9996\u5148\u9700\u8981\u5bf9\u7ec6\u80de\u4fee\u590dDNA\u53cc\u94fe\u65ad\u88c2\uff08DSB\uff09\u7684\u4e3b\u8981\u901a\u8def\u6709\u4e00\u4e2a\u6e05\u6670\u7684\u8ba4\u8bc6\u3002DSB\u662f\u6700\u5177\u7ec6\u80de\u6bd2\u6027\u7684DNA\u635f\u4f24\u7c7b\u578b\uff0c\u5176\u4fee\u590d\u673a\u5236\u7684\u7cbe\u786e\u9009\u62e9\u548c\u6267\u884c\u5bf9\u7ef4\u6301\u57fa\u56e0\u7ec4\u7a33\u5b9a\u81f3\u5173\u91cd\u8981\u3002<\/p>\n\n\n\n

    A. DNA\u53cc\u94fe\u65ad\u88c2\uff08DSB\uff09\u6982\u8ff0<\/h3>\n\n\n\n

    DSB\u662f\u6307DNA\u53cc\u87ba\u65cb\u7684\u4e24\u6761\u94fe\u5728\u540c\u4e00\u4f4d\u7f6e\u6216\u90bb\u8fd1\u4f4d\u7f6e\u53d1\u751f\u65ad\u88c2\uff0c\u5b83\u4f1a\u76f4\u63a5\u5207\u65ad\u67d3\u8272\u4f53 11<\/sup>\u3002\u5982\u679cDSB\u672a\u80fd\u4fee\u590d\u6216\u88ab\u9519\u8bef\u4fee\u590d\uff0c\u5c06\u5bfc\u81f4\u707e\u96be\u6027\u540e\u679c\uff0c\u5305\u62ec\u57fa\u56e0\u7a81\u53d8\u3001\u67d3\u8272\u4f53\u91cd\u6392\uff08\u5982\u6613\u4f4d\uff09\u3001\u57fa\u56e0\u4fe1\u606f\u4e22\u5931\u4e43\u81f3\u7ec6\u80de\u6b7b\u4ea1 13<\/sup>\u3002DSB\u53ef\u7531\u5916\u6e90\u6027\u56e0\u7d20\uff08\u5982\u7535\u79bb\u8f90\u5c04\u3001\u5316\u7597\u836f\u7269\uff09\u6216\u5185\u6e90\u6027\u8fc7\u7a0b\uff08\u5982\u6c27\u5316\u5e94\u6fc0\u3001\u590d\u5236\u53c9\u584c\u9677\uff09\u4ea7\u751f 14<\/sup>\u3002\u56e0\u6b64\uff0c\u7ec6\u80de\u8fdb\u5316\u51fa\u4e86\u4e00\u5957\u590d\u6742\u7684DDR\u7f51\u7edc\uff0c\u4ee5\u9ad8\u6548\u3001\u51c6\u786e\u5730\u4fee\u590dDSB\uff0c\u4ece\u800c\u4fdd\u62a4\u57fa\u56e0\u7ec4\u7684\u5b8c\u6574\u6027\uff0c\u9632\u6b62\u764c\u75c7\u7b49\u75be\u75c5\u7684\u53d1\u751f 13<\/sup>\u3002<\/p>\n\n\n\n

    B. \u975e\u540c\u6e90\u672b\u7aef\u8fde\u63a5\uff08NHEJ\uff09\u901a\u8def<\/h3>\n\n\n\n

    \u975e\u540c\u6e90\u672b\u7aef\u8fde\u63a5\uff08NHEJ\uff09\u662f\u54fa\u4e73\u52a8\u7269\u7ec6\u80de\u4e2d\u4fee\u590dDSB\u7684\u4e3b\u8981\u9014\u5f84\uff0c\u5b83\u5728\u7ec6\u80de\u5468\u671f\u7684\u6240\u6709\u9636\u6bb5\uff08G1\u3001S\u548cG2\/M\u671f\uff09\u90fd\u4fdd\u6301\u6d3b\u8dc3 12<\/sup>\u3002NHEJ\u901a\u8def\u53ef\u4ee5\u76f4\u63a5\u5c06\u65ad\u88c2\u7684DNA\u672b\u7aef\u8fdb\u884c\u8fde\u63a5\uff0c\u51e0\u4e4e\u4e0d\u9700\u8981\u6216\u5b8c\u5168\u4e0d\u4f9d\u8d56\u4e8e\u5e8f\u5217\u540c\u6e90\u6027\u3002\u8be5\u901a\u8def\u7684\u6838\u5fc3\u53c2\u4e0e\u8005\u5305\u62ec\uff1aKu70\/80\u5f02\u6e90\u4e8c\u805a\u4f53\uff08\u4f5c\u4e3aDSB\u7684\u4f20\u611f\u5668\uff0c\u7ed3\u5408\u5e76\u4fdd\u62a4DNA\u672b\u7aef\uff09\u3001DNA\u4f9d\u8d56\u6027\u86cb\u767d\u6fc0\u9176\u50ac\u5316\u4e9a\u57fa\uff08DNA-PKcs\uff09\u3001\u6838\u9178\u9176Artemis\uff08\u52a0\u5de5\u4fee\u526a\u672b\u7aef\uff09\u3001DNA\u805a\u5408\u9176\u03bc\u548c\u03bb\uff08\u586b\u8865\u7f3a\u53e3\uff09\uff0c\u4ee5\u53ca\u6700\u7ec8\u6267\u884c\u8fde\u63a5\u6b65\u9aa4\u7684XRCC4-DNA\u8fde\u63a5\u9176IV\uff08Ligase IV\uff09\u590d\u5408\u7269 13<\/sup>\u3002\u7531\u4e8e\u5176\u76f4\u63a5\u8fde\u63a5\u7684\u7279\u6027\uff0cNHEJ\u672c\u8d28\u4e0a\u662f\u6613\u9519\u7684\uff0c\u5e38\u5e38\u5728\u4fee\u590d\u4f4d\u70b9\u5f15\u5165\u5c0f\u7684\u63d2\u5165\u6216\u5220\u9664\uff08indels\uff0912<\/sup>\u3002<\/p>\n\n\n\n

    C. \u540c\u6e90\u91cd\u7ec4\uff08HR\uff09\u901a\u8def<\/h3>\n\n\n\n

    \u540c\u6e90\u91cd\u7ec4\uff08HR\uff09\u662f\u4e00\u79cd\u9ad8\u4fdd\u771f\u5ea6\u7684DSB\u4fee\u590d\u901a\u8def\uff0c\u5b83\u5229\u7528\u5b8c\u6574\u7684\u59d0\u59b9\u67d3\u8272\u5355\u4f53\u6216\u540c\u6e90\u67d3\u8272\u4f53\u4f5c\u4e3a\u6a21\u677f\uff0c\u7cbe\u786e\u5730\u6062\u590d\u65ad\u88c2\u4f4d\u70b9\u539f\u6709\u7684DNA\u5e8f\u5217 12<\/sup>\u3002\u7531\u4e8e\u9700\u8981\u6a21\u677f\uff0cHR\u901a\u8def\u4e3b\u8981\u5728\u7ec6\u80de\u5468\u671f\u7684S\u671f\u548cG2\u671f\u6d3b\u8dc3\uff0c\u56e0\u4e3a\u6b64\u65f6\u59d0\u59b9\u67d3\u8272\u5355\u4f53\u5df2\u7ecf\u590d\u5236\u5e76\u5b58\u5728 12<\/sup>\u3002HR\u901a\u8def\u7684\u5173\u952e\u86cb\u767d\u5305\u62ecMRE11-RAD50-NBS1\uff08MRN\uff09\u590d\u5408\u7269\uff08\u542f\u52a8\u672b\u7aef\u5207\u9664\uff0c\u4ea7\u751f3’\u5355\u94fe\u60ac\u5782\uff09\u3001RAD51\uff08\u4ecb\u5bfc\u94fe\u5165\u4fb5\u548c\u540c\u6e90\u5e8f\u5217\u641c\u7d22\uff09\u4ee5\u53caBRCA1\/BRCA2\uff08\u4fc3\u8fdbRAD51\u52a0\u8f7d\u5230DNA\u4e0a\uff0911<\/sup>\u3002<\/p>\n\n\n\n

    D. \u66ff\u4ee3\u6027\u672b\u7aef\u8fde\u63a5\uff08Alt-EJ\uff09\u53ca\u5176\u4ed6\u4fee\u590d\u901a\u8def<\/h3>\n\n\n\n

    \u5f53\u7ecf\u5178\u7684NHEJ\u6216HR\u901a\u8def\u53d7\u635f\u6216\u65e0\u6cd5\u8fdb\u884c\u65f6\uff0c\u7ec6\u80de\u53ef\u4ee5\u542f\u7528\u4e00\u4e9b\u5907\u7528\u7684\u3001\u901a\u5e38\u66f4\u6613\u9519\u7684\u4fee\u590d\u9014\u5f84\uff0c\u5982\u5fae\u540c\u6e90\u4ecb\u5bfc\u7684\u672b\u7aef\u8fde\u63a5\uff08MMEJ\uff09\u6216\u66ff\u4ee3\u6027NHEJ\uff08A-NHEJ\uff0914<\/sup>\u3002\u8fd9\u4e9b\u901a\u8def\u5229\u7528\u65ad\u88c2\u672b\u7aef\u5b58\u5728\u7684\u77ed\u5fae\u540c\u6e90\u5e8f\u5217\uff08\u51e0\u4e2a\u6838\u82f7\u9178\uff09\u8fdb\u884c\u9519\u4f4d\u9000\u706b\uff0c\u7136\u540e\u5207\u9664\u4e0d\u5339\u914d\u7684\u60ac\u5782\u5e76\u8fdb\u884c\u8fde\u63a5 20<\/sup>\u3002\u8fd9\u4e9b\u66ff\u4ee3\u6027\u901a\u8def\u867d\u7136\u80fd\u907f\u514d\u67d3\u8272\u4f53\u65ad\u88c2\u5e26\u6765\u7684\u81f4\u547d\u540e\u679c\uff0c\u4f46\u5176\u4fee\u590d\u8fc7\u7a0b\u5f80\u5f80\u4f34\u968f\u7740\u5927\u7247\u6bb5\u7684\u5220\u9664\u6216\u67d3\u8272\u4f53\u6613\u4f4d\uff0c\u662f\u5bfc\u81f4\u57fa\u56e0\u7ec4\u4e0d\u7a33\u5b9a\u7684\u91cd\u8981\u6765\u6e90 14<\/sup>\u3002<\/p>\n\n\n\n

    \u88681\uff1a\u4e3b\u8981DNA\u53cc\u94fe\u65ad\u88c2\u4fee\u590d\u901a\u8def\u7684\u6bd4\u8f83<\/strong><\/p>\n\n\n\n

    \u7279\u5f81<\/td>\u7ecf\u5178\u975e\u540c\u6e90\u672b\u7aef\u8fde\u63a5 (c-NHEJ)<\/td>\u540c\u6e90\u91cd\u7ec4 (HR)<\/td>\u5fae\u540c\u6e90\u4ecb\u5bfc\u7684\u672b\u7aef\u8fde\u63a5 (MMEJ\/Alt-EJ)<\/td><\/tr><\/thead>
    \u6838\u5fc3\u86cb\u767d\u56e0\u5b50<\/strong><\/td>Ku70\/80, DNA-PKcs, Artemis, XRCC4-Ligase IV 13<\/sup><\/td>MRE11, RAD51, BRCA1, BRCA2 11<\/sup><\/td>PARP1, MRE11, Ligase III\/Ligase I 20<\/sup><\/td><\/tr>
    \u6a21\u677f\u9700\u6c42<\/strong><\/td>\u65e0 19<\/sup><\/td>\u59d0\u59b9\u67d3\u8272\u5355\u4f53\/\u540c\u6e90\u67d3\u8272\u4f53 12<\/sup><\/td>\u65e0 (\u5229\u7528\u672b\u7aef\u5fae\u540c\u6e90\u5e8f\u5217) 17<\/sup><\/td><\/tr>
    \u4fdd\u771f\u5ea6<\/strong><\/td>\u4f4e (\u6613\u9519) 12<\/sup><\/td>\u9ad8 (\u4fdd\u771f) 12<\/sup><\/td>\u975e\u5e38\u4f4e (\u9ad8\u5ea6\u81f4\u7a81\u53d8) 14<\/sup><\/td><\/tr>
    \u4e3b\u8981\u7ec6\u80de\u5468\u671f<\/strong><\/td>G1, S, G2\/M (\u5168\u7a0b\u6d3b\u8dc3) 12<\/sup><\/td>S, G2 12<\/sup><\/td>\u5f53c-NHEJ\/HR\u53d7\u635f\u65f6\u6fc0\u6d3b 14<\/sup><\/td><\/tr>
    \u5178\u578b\u7a81\u53d8\u7279\u5f81<\/strong><\/td>\u5c0f\u7684\u63d2\u5165\u6216\u5220\u9664 (Indels) 19<\/sup><\/td>\u65e0\u7a81\u53d8\u6216\u57fa\u56e0\u8f6c\u6362 12<\/sup><\/td>\u4f34\u968f\u5fae\u540c\u6e90\u5e8f\u5217\u7684\u5927\u7247\u6bb5\u5220\u9664 22<\/sup><\/td><\/tr><\/tbody><\/table><\/figure>\n\n\n\n

    \u7ec6\u80de\u5185\u591a\u79cdDSB\u4fee\u590d\u901a\u8def\u5e76\u5b58\u4e14\u76f8\u4e92\u7ade\u4e89\u7684\u73b0\u8c61\uff0c\u63ed\u793a\u4e86\u4e00\u4e2a\u6df1\u523b\u7684\u751f\u7269\u5b66\u539f\u7406\uff1a\u7ec6\u80de\u7684\u9996\u8981\u76ee\u6807\u5e76\u975e\u603b\u662f\u8ffd\u6c42\u5b8c\u7f8e\u7684\u4fdd\u771f\u5ea6\uff0c\u800c\u662f\u5728\u7279\u5b9a\u60c5\u5883\u4e0b\u7684\u751f\u5b58\u3002\u4fee\u590d\u901a\u8def\u7684\u9009\u62e9\u662f\u4e00\u4e2a\u53d7\u5230\u7ec6\u80de\u5468\u671f\u3001DNA\u65ad\u88c2\u672b\u7aef\u7ed3\u6784\u7b49\u591a\u79cd\u56e0\u7d20\u7cbe\u5bc6\u8c03\u63a7\u7684\u51b3\u7b56\u8fc7\u7a0b\u3002\u4f8b\u5982\uff0c\u5728G1\u671f\uff0c\u901a\u8fc7NHEJ\u5feb\u901f\u4fee\u590dDSB\u4ee5\u9632\u6b62\u67d3\u8272\u4f53\u4e22\u5931\uff0c\u5373\u4fbf\u4ed8\u51fa\u5f15\u5165\u5c0f\u7a81\u53d8\u7684\u4ee3\u4ef7\uff0c\u4e5f\u4f18\u4e8e\u643a\u5e26\u672a\u4fee\u590d\u65ad\u88c2\u8fdb\u5165S\u671f\u6240\u9762\u4e34\u7684\u81f4\u547d\u98ce\u9669\u3002\u8fd9\u79cd\u5bf9\u4fee\u590d\u7b56\u7565\u7684\u201c\u5b9e\u7528\u4e3b\u4e49\u201d\u9009\u62e9\uff0c\u8868\u660e\u6574\u4e2aDDR\u7cfb\u7edf\u5177\u6709\u9ad8\u5ea6\u7684\u9002\u5e94\u6027\u548c\u7075\u6d3b\u6027\u3002\u6b63\u662f\u8fd9\u79cd\u7075\u6d3b\u6027\uff0c\u4e3a\u975e\u7ecf\u5178\u7684\u3001\u5177\u6709\u7279\u6b8a\u529f\u80fd\u7684\u4fee\u590d\u56e0\u5b50\uff08\u5982RTCB\uff09\u7684\u5b58\u5728\u548c\u53c2\u4e0e\u63d0\u4f9b\u4e86\u6982\u5ff5\u7a7a\u95f4\u3002\u5982\u679cRTCB\u5728DNA\u4fee\u590d\u4e2d\u786e\u6709\u5176\u804c\uff0c\u5b83\u53ef\u80fd\u5e76\u975e\u4f5c\u4e3aNHEJ\u6216HR\u7684\u6838\u5fc3\u7ec4\u5206\uff0c\u800c\u662f\u626e\u6f14\u4e00\u4e2a\u201c\u4e13\u5bb6\u201d\u89d2\u8272\uff0c\u4e13\u95e8\u5904\u7406\u67d0\u79cd\u7f55\u89c1\u7684DNA\u672b\u7aef\u635f\u4f24\uff0c\u6216\u662f\u5728\u7279\u5b9a\u5e94\u6fc0\u6761\u4ef6\u4e0b\u88ab\u6fc0\u6d3b\uff0c\u53c2\u4e0e\u4e00\u6761\u7279\u6b8a\u7684\u4fee\u590d\u652f\u8def\u3002<\/p>\n\n\n\n

    III. \u76f4\u63a5\u7684\u673a\u5236\u91cd\u53e0\uff1aRTCB\u8fde\u63a5\u9176\u5bb6\u65cf\u6f5c\u5728\u7684DNA\u4fee\u9970\u6d3b\u6027<\/h2>\n\n\n\n

    \u5c3d\u7ba1RTCB\u5728\u771f\u6838\u751f\u7269\u4e2d\u7684\u4e3b\u8981\u5e95\u7269\u662fRNA\uff0c\u4f46\u6765\u81ea\u539f\u6838\u751f\u7269\u7684\u7814\u7a76\u63d0\u4f9b\u4e86\u5176\u4e0eDNA\u4ee3\u8c22\u76f4\u63a5\u76f8\u5173\u7684\u6700\u6709\u529b\u8bc1\u636e\uff0c\u63ed\u793a\u4e86RTCB\u5bb6\u65cf\u6210\u5458\u8d85\u8d8aRNA\u8fde\u63a5\u7684\u60ca\u4eba\u9176\u5b66\u6f5c\u529b\u3002<\/p>\n\n\n\n

    A. \u6765\u81ea\u7ec6\u83cc\u7cfb\u7edf\u7684\u8bc1\u636e\uff1a\u5927\u80a0\u6746\u83cc\u4e0e\u9ec4\u8272\u7c98\u7403\u83cc\u7684RtcB<\/h3>\n\n\n\n

    \u5bf9\u7ec6\u83ccRtcB\u540c\u6e90\u7269\u7684\u7814\u7a76\u9996\u6b21\u63ed\u793a\u4e86\u5176\u4f5c\u7528\u4e8eDNA\u5e95\u7269\u7684\u975e\u5178\u578b\u6d3b\u6027 8<\/sup>\u3002\u7814\u7a76\u53d1\u73b0\uff0c\u5927\u80a0\u6746\u83cc\uff08<\/p>\n\n\n\n

    E. coli<\/em>\uff09\u7684RtcB\u80fd\u591f\u5c06\u5176\u5171\u4ef7\u7ed3\u5408\u7684GMP\u4e2d\u95f4\u4f53\uff0c\u8f6c\u79fb\u5230\u5355\u94feDNA\u5206\u5b50\u76843′-\u78f7\u9178\u672b\u7aef 8<\/sup>\u3002<\/p>\n\n\n\n

    \u529f\u80fd\u591a\u6837\u6027\u7684\u8bc1\u636e\u5728\u5bf9\u9ec4\u8272\u7c98\u7403\u83cc\uff08Myxococcus xanthus<\/em>\uff09\u7684\u7814\u7a76\u4e2d\u8868\u73b0\u5f97\u66f4\u4e3a\u7a81\u51fa\u3002\u8be5\u83cc\u682a\u62e5\u6709\u516d\u4e2aRtcB\u65c1\u7cfb\u540c\u6e90\u7269\uff0c\u5bf9\u5176\u8fdb\u884c\u5206\u6790\u540e\u53d1\u73b0\uff0cRtcB1\u548cRtcB2\u662f\u6709\u6548\u7684RNA\u8fde\u63a5\u9176\uff0c\u800cRtcB3\u7684RNA\u8fde\u63a5\u6d3b\u6027\u5374\u975e\u5e38\u5fae\u5f31\uff0c\u4f46\u5b83\u5728\u4fee\u9970DNA\u672b\u7aef\u65b9\u9762\u8868\u73b0\u51fa\u6781\u9ad8\u7684\u6548\u7387 8<\/sup>\u3002\u8fd9\u79cd\u529f\u80fd\u4e0a\u7684\u5206\u5316\u8868\u660e\uff0cRTCB\u5bb6\u65cf\u7684\u67d0\u4e9b\u6210\u5458\u53ef\u80fd\u5728\u8fdb\u5316\u8fc7\u7a0b\u4e2d\u7279\u5316\u51fa\u4e86\u5904\u7406DNA\u5e95\u7269\u7684\u80fd\u529b\u3002<\/p>\n\n\n\n

    B. DNA 3′-\u78f7\u9178\u672b\u7aef\u7684\u201c\u52a0\u5e3d\u201d\uff1a\u4e00\u79cd\u65b0\u9896\u7684\u4fee\u9970<\/h3>\n\n\n\n

    RTCB\u5bf9DNA\u7684\u4e3b\u8981\u4fee\u9970\u6d3b\u6027\u662f\u5728\u51763’\u672b\u7aef\u52a0\u4e0a\u4e00\u4e2a\u201c\u5e3d\u5b50\u201d\uff0c\u5f62\u6210\u4e00\u4e2a\u5316\u5b66\u6027\u8d28\u7a33\u5b9a\u7684DNA-3’ppG\u7ed3\u6784\uff08\u5373\u4e00\u4e2a\u9e1f\u82f7\u9178\u57fa\u56e2\u901a\u8fc7\u7126\u78f7\u9178\u952e\u8fde\u63a5\u5230DNA\u76843’\u672b\u7aef\uff098<\/sup>\u3002\u8fd9\u79cd\u201c\u52a0\u5e3d\u201d\u53cd\u5e94\u4ece\u6839\u672c\u4e0a\u6539\u53d8\u4e86DNA\u672b\u7aef\u7684\u5316\u5b66\u6027\u8d28\uff0c\u4f7f\u5176\u65e0\u6cd5\u88ab\u6807\u51c6\u7684DNA\u805a\u5408\u9176\u6216\u8fde\u63a5\u9176\u8bc6\u522b\u3002\u8fd9\u6697\u793a\u8be5\u4fee\u9970\u53ef\u80fd\u5177\u6709\u4e24\u79cd\u6f5c\u5728\u529f\u80fd\uff1a\u4e00\u662f\u4fdd\u62a4DNA\u672b\u7aef\u514d\u53d7\u6838\u9178\u5916\u5207\u9176\u7684\u964d\u89e3\uff0c\u4e8c\u662f\u5bf9\u5176\u8fdb\u884c\u6807\u8bb0\uff0c\u4ee5\u5f15\u5bfc\u7279\u5b9a\u7684\u4e0b\u6e38\u4fee\u590d\u8fc7\u7a0b\u3002\u503c\u5f97\u6ce8\u610f\u7684\u662f\uff0c<\/p>\n\n\n\n

    M. xanthus<\/em> RtcB3\u8fd8\u8868\u73b0\u51fa\u4e00\u79cd\u66f4\u4e3a\u72ec\u7279\u7684\u6d3b\u6027\uff0c\u5373\u80fd\u591f\u5bf9DNA\u548cRNA\u76845′-\u78f7\u9178\u672b\u7aef\u8fdb\u884c\u52a0\u5e3d\uff0c\u5f62\u6210GppDNA\u548cGppRNA\uff0c\u8fd9\u662f\u5728\u5927\u80a0\u6746\u83ccRtcB\u4e2d\u672a\u66fe\u53d1\u73b0\u7684\u65b0\u529f\u80fd 8<\/sup>\u3002<\/p>\n\n\n\n

    C. RTCB\u52a0\u5e3d\u7684DNA\u53ef\u88abDNA\u4fee\u590d\u9176Aprataxin\u5904\u7406<\/h3>\n\n\n\n

    \u8fd9\u4e00\u53d1\u73b0\u4e2d\u6700\u5177\u542f\u53d1\u6027\u7684\u4e00\u73af\u662f\uff0c\u7531RtcB3\u5728DNA\u4e0a\u5b89\u88c5\u7684GppDNA\u5e3d\u5b50\uff0c\u53ef\u4ee5\u88ab\u4eba\u7c7bDNA\u4fee\u590d\u9176Aprataxin\uff08APTX\uff09\u7279\u5f02\u6027\u5730\u53bb\u9664 8<\/sup>\u3002Aprataxin\u80fd\u591f\u201c\u8131\u5e3d\u201d\uff0c\u5c06DNA-3’ppG\u672b\u7aef\u8fd8\u539f\u4e3a\u4e00\u4e2a\u53ef\u88ab\u8fde\u63a5\u76843′-\u78f7\u9178\u3002<\/p>\n\n\n\n

    Aprataxin\u672c\u8eab\u5c31\u662fDDR\u5de5\u5177\u7bb1\u4e2d\u7684\u4e00\u4e2a\u5df2\u77e5\u6210\u5458\uff0c\u5176\u7ecf\u5178\u529f\u80fd\u662f\u89e3\u51b3\u5931\u8d25\u7684DNA\u8fde\u63a5\u4e8b\u4ef6\uff0c\u7279\u522b\u662f\u53bb\u9664DNA 5’\u672b\u7aef\u7531\u4e8eDNA\u8fde\u63a5\u9176I\u4e2d\u6b62\u53cd\u5e94\u800c\u7559\u4e0b\u7684AMP\u57fa\u56e2\u3002\u5b83\u80fd\u591f\u8bc6\u522b\u5e76\u4f5c\u7528\u4e8e\u4e00\u4e2a\u975e\u5178\u578b\u76843′-GMP\u5e3d\u5b50\uff0c\u8fd9\u662f\u4e00\u4e2a\u5168\u65b0\u7684\u53d1\u73b0\uff0c\u5e76\u5f3a\u70c8\u6697\u793a\u4e86RTCB\u4ecb\u5bfc\u7684DNA\u4fee\u9970\u4e0e\u54fa\u4e73\u52a8\u7269\u7684DNA\u4fee\u590d\u901a\u8def\u4e4b\u95f4\u5b58\u5728\u7740\u76f4\u63a5\u7684\u529f\u80fd\u8054\u7cfb\u3002<\/p>\n\n\n\n

    RTCB-Aprataxin\u8f74\u7684\u5b58\u5728\uff0c\u63cf\u7ed8\u4e86\u4e00\u4e2a\u6f5c\u5728\u7684\u3001\u5b8c\u6574\u7684\u3001\u65b0\u9896\u7684DNA\u672b\u7aef\u52a0\u5de5\u901a\u8def\u3002\u8fd9\u4e0d\u4ec5\u4ec5\u662f\u4e00\u79cd\u9176\u5b66\u4e0a\u7684\u5de7\u5408\uff0c\u800c\u662f\u4e00\u4e2a\u529f\u80fd\u6a21\u5757\u3002\u5728\u8fd9\u4e2a\u6a21\u5757\u4e2d\uff0cRTCB\u626e\u6f14\u4e86\u201c\u4fee\u9970\u8005\u201d\u6216\u201c\u4fdd\u62a4\u8005\u201d\u7684\u89d2\u8272\uff0c\u5904\u7406\u4e00\u4e2a\u6709\u95ee\u9898\u76843′-\u78f7\u9178\u672b\u7aef\uff1b\u800cAprataxin\u5219\u626e\u6f14\u4e86\u201c\u6062\u590d\u8005\u201d\u7684\u89d2\u8272\uff0c\u4e3a\u540e\u7eed\u7684\u7ecf\u5178\u4fee\u590d\u6b65\u9aa4\u505a\u51c6\u5907\u3002\u8fd9\u4e2a\u4e24\u6b65\u8fc7\u7a0b\u53ef\u80fd\u662f\u4e00\u79cd\u4e13\u95e8\u7684\u673a\u5236\uff0c\u7528\u4e8e\u5904\u7406\u7531\u7279\u5b9a\u7c7b\u578b\u635f\u4f24\uff08\u4f8b\u5982\u67d0\u4e9b\u5316\u5b66\u836f\u7269\u6216\u5185\u6e90\u6027\u4ee3\u8c22\u4ea7\u7269\uff09\u4ea7\u751f\u7684\u3001\u5e26\u67093′-\u78f7\u9178\u672b\u7aef\u7684DNA\u65ad\u88c2\u3002\u8fd9\u7c7b\u672b\u7aef\u5bf9\u4e8e\u6807\u51c6\u7684DNA\u805a\u5408\u9176\u548c\u8fde\u63a5\u9176\u800c\u8a00\u662f\u7cdf\u7cd5\u7684\u5e95\u7269\u3002<\/p>\n\n\n\n

    \u4e00\u4e2a\u4e13\u95e8\u7684\u201c\u64e6\u9664\u5668\u201d\uff08Aprataxin\uff09\u7684\u5b58\u5728\uff0c\u5f3a\u70c8\u6697\u793a\u4e86\u201c\u4e66\u5199\u5668\u201d\uff08RTCB\uff09\u6267\u884c\u7684\u662f\u5177\u6709\u751f\u7269\u5b66\u610f\u4e49\u7684\u529f\u80fd\u3002\u7ec6\u80de\u8fdb\u5316\u51fa\u4e86\u4e00\u79cd\u673a\u5236\u6765\u9006\u8f6c\u8fd9\u79cd\u7279\u5b9a\u7684DNA\u4fee\u9970\uff0c\u8bf4\u660e\u8fd9\u79cd\u4fee\u9970\u5728\u7ec6\u80de\u751f\u547d\u6d3b\u52a8\u4e2d\u786e\u5b9e\u4f1a\u53d1\u751f\u4e14\u9700\u8981\u88ab\u8c03\u63a7\u3002\u636e\u6b64\u53ef\u4ee5\u63a8\u65ad\uff0c\u8be5\u6a21\u5757\u53ef\u80fd\u4f5c\u4e3a\u4e00\u79cd\u7279\u6b8a\u7684DNA\u672b\u7aef\u4fdd\u62a4\u548c\u52a0\u5de5\u5f62\u5f0f\u53d1\u6325\u4f5c\u7528\u3002\u5728DNA\u65ad\u88c2\u4ea7\u751f3′-\u78f7\u9178\u672b\u7aef\u7684\u60c5\u5883\u4e0b\uff0cRTCB\u53ef\u4ee5\u5bf9\u5176\u8fdb\u884c\u52a0\u5e3d\uff0c\u4ee5\u9632\u6b62\u6838\u9178\u5916\u5207\u9176\u7684\u964d\u89e3\u6216\u9519\u8bef\u7684\u8fde\u63a5\uff0c\u5f62\u6210\u4e00\u4e2a\u7a33\u5b9a\u7684\u201c\u7b49\u5f85\u201d\u72b6\u6001\u3002\u968f\u540e\uff0cAprataxin\u53ef\u80fd\u88ab\u62db\u52df\u6765\u53bb\u9664\u5e3d\u5b50\uff0c\u66b4\u9732\u51fa\u4e00\u4e2a\u5e72\u51c0\u76843′-\u78f7\u9178\u672b\u7aef\uff0c\u8be5\u672b\u7aef\u53ef\u8fdb\u4e00\u6b65\u88ab\u78f7\u9178\u9176\uff08\u5982PNKP\uff09\u5904\u7406\u62103′-\u7f9f\u57fa\uff0c\u6700\u7ec8\u7531\u7ecf\u5178\u7684DNA\u8fde\u63a5\u9176\uff08\u5982Ligase IV\u6216Ligase III\uff09\u8fdb\u884c\u8fde\u63a5\u3002\u8fd9\u4e00\u6a21\u578b\u5c06RTCB\u5b9a\u4f4d\u4e3a\u7279\u6b8aNHEJ\u6216\u78b1\u57fa\u5207\u9664\u4fee\u590d\uff08BER\uff09\u4e9a\u901a\u8def\u4e2d\u7684\u4e00\u4e2a\u6f5c\u5728\u4e0a\u6e38\u56e0\u5b50\u3002\u7136\u800c\uff0c\u4e00\u4e2a\u81f3\u5173\u91cd\u8981\u7684\u3001\u60ac\u800c\u672a\u51b3\u7684\u95ee\u9898\u662f\uff0c\u8fd9\u79cd\u6d3b\u6027\u662f\u5426\u5728\u540e\u751f\u52a8\u7269\u7ec6\u80de\u4e2d\u771f\u5b9e\u5b58\u5728\u5e76\u53d1\u6325\u529f\u80fd\u3002<\/p>\n\n\n\n

    IV. \u901a\u8fc7\u86cb\u767d\u7f51\u7edc\u7684\u95f4\u63a5\u5173\u8054\uff1atRNA\u8fde\u63a5\u9176\u590d\u5408\u7269\u53ca\u5176\u4e0eDNA\u4fee\u590d\u673a\u5668\u7684\u8054\u7cfb<\/h2>\n\n\n\n

    \u5728\u9ad8\u7b49\u771f\u6838\u751f\u7269\u4e2d\uff0c\u8fde\u63a5RTCB\u4e0eDDR\u7684\u6700\u6709\u529b\u8bc1\u636e\u5e76\u975e\u6765\u81ea\u5176\u76f4\u63a5\u7684\u9176\u6d3b\u6027\uff0c\u800c\u662f\u901a\u8fc7\u5176\u6240\u5728\u7684\u86cb\u767d\u8d28\u590d\u5408\u7269\u7684\u7269\u7406\u76f8\u4e92\u4f5c\u7528\u3002<\/p>\n\n\n\n

    A. \u540e\u751f\u52a8\u7269tRNA\u8fde\u63a5\u9176\u590d\u5408\u7269\uff08tRNA-LC\uff09\u7684\u7ed3\u6784<\/h3>\n\n\n\n

    \u5728\u540e\u751f\u52a8\u7269\u4e2d\uff0cRTCB\u5e76\u975e\u72ec\u7acb\u53d1\u6325\u529f\u80fd\uff0c\u800c\u662f\u4f5c\u4e3a\u4e00\u4e2a\u4e94\u4e9a\u57fatRNA\u8fde\u63a5\u9176\u590d\u5408\u7269\uff08tRNA-LC\uff09\u7684\u50ac\u5316\u6838\u5fc3 3<\/sup>\u3002\u8be5\u590d\u5408\u7269\u7684\u5176\u4ed6\u56db\u4e2a\u6210\u5458\u5305\u62ec\uff1aDEAD-box RNA\u89e3\u65cb\u9176DDX1\uff0c\u4ee5\u53ca\u53e6\u5916\u4e09\u79cd\u529f\u80fd\u5c1a\u4e0d\u5b8c\u5168\u6e05\u695a\u7684\u86cb\u767d\uff1aRTRAF\uff08\u4e5f\u79f0\u4e3aCLE\uff09\u3001FAM98B\u548cAshwin\uff08ASW\uff093<\/sup>\u3002\u8fd9\u79cd\u591a\u4e9a\u57fa\u7684\u590d\u5408\u7269\u7ed3\u6784\u8868\u660e\uff0cRTCB\u7684\u6d3b\u6027\u53d7\u5230\u5176\u86cb\u767d\u4f19\u4f34\u7684\u4e25\u5bc6\u8c03\u63a7\uff0c\u5e76\u4e0e\u5176\u4ed6\u7ec6\u80de\u529f\u80fd\u7d27\u5bc6\u6574\u5408\u3002<\/p>\n\n\n\n

    B. DDX1\u4e9a\u57fa\uff1a\u8fde\u63a5RNA\u8fde\u63a5\u4e0eDNA\u4fee\u590d\u7684\u6865\u6881<\/h3>\n\n\n\n

    DDX1\u662ftRNA-LC\u7684\u91cd\u8981\u7ec4\u6210\u90e8\u5206\uff0c\u5176ATP\u4f9d\u8d56\u7684RNA\u89e3\u65cb\u9176\u6d3b\u6027\u5bf9\u4e8eRTCB\u4ecb\u5bfc\u7684RNA\u8fde\u63a5\u53cd\u5e94\u8fbe\u5230\u6700\u9ad8\u6548\u7387\u662f\u5fc5\u9700\u7684 3<\/sup>\u3002\u81f3\u5173\u91cd\u8981\u7684\u662f\uff0cDDX1\u672c\u8eab\u5728DNA\u53cc\u94fe\u65ad\u88c2\u4fee\u590d\u4e2d\u62e5\u6709\u4e00\u4e2a\u5df2\u5f97\u5230\u5145\u5206\u8bc1\u5b9e\u7684\u3001\u72ec\u7acb\u4e8etRNA-LC\u7684\u529f\u80fd 3<\/sup>\u3002<\/p>\n\n\n\n

    \u6b64\u5916\uff0ctRNA-LC\u7684\u7ec4\u5206\u8868\u73b0\u51fa\u52a8\u6001\u7684\u76f8\u4e92\u4f5c\u7528\u6a21\u5f0f\u3002\u4f8b\u5982\uff0cRTCB\u3001DDX1\u548cRTRAF\u88ab\u53d1\u73b0\u5728\u795e\u7ecf\u5143\u7684RNA\u8fd0\u8f93\u590d\u5408\u7269\u4e2d\u5171\u540c\u5b58\u5728\uff0c\u5e76\u4e14\u5728HEK293T\u7ec6\u80de\u4e2d\u4e0emRNA\u76845’\u5e3d\u5b50\u7ed3\u6784\u76f8\u5173\u8054 4<\/sup>\u3002\u8fd9\u8868\u660e\u8fd9\u4e9b\u86cb\u767d\u4e4b\u95f4\u7684\u76f8\u4e92\u4f5c\u7528\u662f\u52a8\u6001\u7684\uff0c\u5e76\u4e14\u53ef\u80fd\u53c2\u4e0e\u4e86\u9664tRNA\u526a\u63a5\u4e4b\u5916\u7684\u591a\u79cd\u7ec6\u80de\u8fc7\u7a0b\u3002DDX1\u7684\u53cc\u91cd\u89d2\u8272\uff0c\u4f7f\u5176\u6210\u4e3a\u8fde\u63a5RNA\u4ee3\u8c22\u548cDNA\u4fee\u590d\u8fd9\u4e24\u4e2a\u6838\u5fc3\u7ec6\u80de\u8fc7\u7a0b\u7684\u7269\u7406\u6865\u6881\u3002<\/p>\n\n\n\n

    \u88682\uff1a\u4eba\u7c7btRNA\u8fde\u63a5\u9176\u590d\u5408\u7269\uff08tRNA-LC\uff09\u7684\u4e9a\u57fa\u53ca\u5176\u529f\u80fd<\/strong><\/p>\n\n\n\n

    \u4e9a\u57fa\u540d\u79f0<\/td>\u5728tRNA-LC\u4e2d\u7684\u6838\u5fc3\u529f\u80fd<\/td>\u5df2\u77e5\u6216\u63a8\u6d4b\u7684tRNA-LC\u5916\u529f\u80fd<\/td><\/tr><\/thead>
    RTCB<\/strong><\/td>\u50ac\u5316\u6838\u5fc3\uff0c\u6267\u884cRNA\u8fde\u63a5\u53cd\u5e94 4<\/sup><\/td>\u5728\u7ec6\u83cc\u4e2d\u53ef\u4fee\u9970DNA\u672b\u7aef 8<\/sup><\/td><\/tr>
    DDX1<\/strong><\/td>ATP\u4f9d\u8d56\u7684RNA\u89e3\u65cb\u9176\uff0c\u589e\u5f3aRTCB\u7684\u8fde\u63a5\u6548\u7387 3<\/sup><\/td>DNA\u53cc\u94fe\u65ad\u88c2\u4fee\u590d<\/strong>\uff0cmicroRNA\u6210\u719f\uff0c\u80f0\u5c9b\u7d20\u7ffb\u8bd1\u8c03\u63a7 3<\/sup><\/td><\/tr>
    RTRAF<\/strong><\/td>\u529f\u80fd\u672a\u77e5\uff0c\u53ef\u80fd\u4e0e\u590d\u5408\u7269\u5b9a\u4f4d\u76f8\u5173 4<\/sup><\/td>\u8f6c\u5f55\u8c03\u63a7\uff0c\u4e0eRNA\u805a\u5408\u9176II\u5171\u5b9a\u4f4d 4<\/sup><\/td><\/tr>
    FAM98B<\/strong><\/td>\u529f\u80fd\u672a\u77e5 4<\/sup><\/td>\u53ef\u4e0eRTRAF\u5f62\u6210\u5f02\u6e90\u4e8c\u805a\u4f53 4<\/sup><\/td><\/tr>
    Ashwin (ASW)<\/strong><\/td>\u529f\u80fd\u672a\u77e5\uff0c\u53ef\u80fd\u6709\u52a9\u4e8e\u590d\u5408\u7269\u7684\u6838\u5b9a\u4f4d 4<\/sup><\/td>\u8c03\u63a7\u975e\u6d32\u722a\u87fe\u80da\u80ce\u53d1\u80b2\u548c\u7ec6\u80de\u5b58\u6d3b 4<\/sup><\/td><\/tr><\/tbody><\/table><\/figure>\n\n\n\n

    tRNA-LC\u7684\u6784\u6210\u63ed\u793a\u4e86\u4e00\u4e2a\u6bd4\u9759\u6001\u9176\u590d\u5408\u7269\u66f4\u4e3a\u590d\u6742\u7684\u56fe\u666f\uff0c\u5b83\u53ef\u80fd\u4f5c\u4e3a\u4e00\u4e2a\u52a8\u6001\u7684\u201c\u4f20\u611f\u5668\u201d\u548c\u201c\u6574\u5408\u5668\u201d\uff0c\u534f\u8c03\u7740\u7ffb\u8bd1\u673a\u5668\u7684\u72b6\u6001\u4e0eDNA\u635f\u4f24\u5e94\u7b54\u3002RTCB\uff08\u4e00\u4e2a\u5173\u952e\u7684RNA\u52a0\u5de5\u9176\uff09\u548cDDX1\uff08\u4e00\u4e2aDDR\u86cb\u767d\uff09\u4e4b\u95f4\u7684\u7269\u7406\u8fde\u63a5\u5f88\u53ef\u80fd\u5e76\u975e\u5076\u7136\u3002\u4e0e\u5176\u5c06\u5b83\u4eec\u89c6\u4e3a\u78b0\u5de7\u5171\u4eab\u4e00\u4e2a\u590d\u5408\u7269\u7684\u3001\u5177\u6709\u72ec\u7acb\u5de5\u4f5c\u7684\u4e24\u4e2a\u86cb\u767d\u8d28\uff0c\u4e00\u4e2a\u66f4\u5408\u7406\u7684\u6a21\u578b\u662f\uff0c\u8be5\u590d\u5408\u7269\u672c\u8eab\u5c31\u662f\u4e00\u4e2a\u4fe1\u53f7\u4e2d\u67a2\u3002<\/p>\n\n\n\n

    \u8fd9\u4e2a\u6a21\u578b\u7684\u903b\u8f91\u63a8\u6f14\u5982\u4e0b\uff1a\u9996\u5148\uff0c\u89c2\u5bdf\u5230RTCB\uff08\u8d1f\u8d23RNA\u6210\u719f\uff09\u548cDDX1\uff08\u8d1f\u8d23DNA\u4fee\u590d\uff09\u5728tRNA-LC\u4e2d\u7269\u7406\u4e0a\u88ab\u6346\u7ed1\u5728\u4e00\u8d77 3<\/sup>\u3002\u5176\u6b21\uff0cDDX1\u7684\u6d3b\u6027\u5f71\u54cdRTCB\u5728\u590d\u5408\u7269\u5185\u7684\u6d3b\u6027\uff0c\u8868\u660e\u5b83\u4eec\u4e4b\u95f4\u5b58\u5728\u529f\u80fd\u4e0a\u7684\u76f8\u4e92\u4f9d\u8d56\uff0c\u800c\u4e0d\u4ec5\u4ec5\u662f\u5171\u5b58 4<\/sup>\u3002\u518d\u8005\uff0c\u8be5\u590d\u5408\u7269\u662f\u52a8\u6001\u7684\uff0c\u5176\u7ec4\u5206\u53ef\u4ee5\u4e0e\u5176\u4ed6\u7ec6\u80de\u673a\u5668\uff08\u5982RNA\u8fd0\u8f93\u9897\u7c92\uff09\u76f8\u4e92\u4f5c\u7528 4<\/sup>\u3002\u7531\u6b64\u53ef\u4ee5\u63a8\u65ad\uff0c\u8be5\u590d\u5408\u7269\u5e76\u975e\u4e00\u4e2a\u9759\u6001\u7684\u3001\u529f\u80fd\u5355\u4e00\u7684\u5b9e\u4f53\uff0c\u5176\u7ec4\u6210\u548c\u5b9a\u4f4d\u53ef\u80fd\u4f1a\u54cd\u5e94\u7ec6\u80de\u4fe1\u53f7\u800c\u6539\u53d8\u3002<\/p>\n\n\n\n

    \u57fa\u4e8e\u6b64\uff0c\u53ef\u4ee5\u63d0\u51fa\u4e00\u4e2a\u5047\u8bbe\uff1a\u5728\u6b63\u5e38\u751f\u7406\u6761\u4ef6\u4e0b\uff0ctRNA-LC\u4e3b\u8981\u53c2\u4e0etRNA\u548cmRNA\u7684\u52a0\u5de5\u3002\u5f53\u7ec6\u80de\u906d\u9047\u57fa\u56e0\u6bd2\u6027\u5e94\u6fc0\u65f6\uff0c\u53ef\u80fd\u4f1a\u53d1\u751f\u4e24\u79cd\u60c5\u51b5\uff1a\uff08a\uff09DDX1\u4ece\u590d\u5408\u7269\u4e2d\u91ca\u653e\u51fa\u6765\uff0c\u4e13\u804c\u53c2\u4e0eDNA\u4fee\u590d\uff0c\u800c\u5b83\u7684\u79bb\u5f00\u672c\u8eab\u53ef\u80fd\u5c31\u662f\u4e00\u4e2a\u4fe1\u53f7\uff0c\u5bfc\u81f4tRNA\u52a0\u5de5\u901f\u7387\u51cf\u6162\uff0c\u4ece\u800c\u8282\u7ea6\u8d44\u6e90\u96c6\u4e2d\u7528\u4e8e\u4fee\u590d\uff1b\uff08b\uff09\u6574\u4e2a\u590d\u5408\u7269\u6216\u5176\u67d0\u4e2a\u4e9a\u578b\u88ab\u62db\u52df\u5230DNA\u635f\u4f24\u4f4d\u70b9\u6216\u7279\u5b9a\u7684\u6838\u5185\u533a\u57df\u3002\u5728\u8fd9\u79cd\u60c5\u5883\u4e0b\uff0cDDX1\u6267\u884c\u5176\u4fee\u590d\u529f\u80fd\uff0c\u800cRTCB\u53ca\u5176\u4ed6\u4e9a\u57fa\u53ef\u80fd\u53d1\u6325\u7ed3\u6784\u6027\u6216\u8c03\u63a7\u6027\u4f5c\u7528\uff0c\u4f8b\u5982\u901a\u8fc7\u4e0e\u635f\u4f24\u4f4d\u70b9\u9644\u8fd1\u7684\u5c40\u90e8RNA\u76f8\u4e92\u4f5c\u7528\u6765\u7a33\u5b9a\u4fee\u590d\u590d\u5408\u4f53\u3002\u8be5\u6a21\u578b\u5c06tRNA-LC\u5b9a\u4f4d\u4e3a\u4e00\u4e2a\u5173\u952e\u8282\u70b9\uff0c\u5e2e\u52a9\u7ec6\u80de\u5728\u5e94\u6fc0\u65f6\u671f\u5e73\u8861\u57fa\u56e0\u7ec4\u7ef4\u62a4\u548c\u86cb\u767d\u8d28\u5408\u6210\u8fd9\u4e24\u4e2a\u76f8\u4e92\u7ade\u4e89\u7684\u6838\u5fc3\u9700\u6c42\u3002<\/p>\n\n\n\n

    V. \u8c03\u63a7\u7f51\u7edc\u4e2d\u7684\u4e32\u6270\uff1a\u57fa\u56e0\u6bd2\u6027\u5e94\u6fc0\u5bf9RTCB\u901a\u8def\u7684\u8bf1\u5bfc<\/h2>\n\n\n\n

    \u9664\u4e86\u7269\u7406\u4e0a\u7684\u8054\u7cfb\uff0cDNA\u635f\u4f24\u4e0eRTCB\u901a\u8def\u4e4b\u95f4\u8fd8\u5b58\u5728\u7740\u529f\u80fd\u6027\u7684\u8c03\u63a7\u8054\u7cfb\uff0c\u5373\u57fa\u56e0\u6bd2\u6027\u5e94\u6fc0\u80fd\u591f\u76f4\u63a5\u8bf1\u5bfcRTCB\u7684\u8868\u8fbe\u3002<\/p>\n\n\n\n

    A. DNA\u635f\u4f24\u8bf1\u5bfc\u7684SOS\u53cd\u5e94\u4e0e\u7ec6\u83ccrtc<\/em>\u64cd\u7eb5\u5b50<\/h3>\n\n\n\n

    \u5728\u6c99\u95e8\u6c0f\u83cc\uff08Salmonella<\/em>\uff09\u7b49\u7ec6\u83cc\u4e2d\uff0c\u7f16\u7801RtcA\u73af\u5316\u9176\u548cRtcB\u8fde\u63a5\u9176\u7684rtcBA<\/em>\u64cd\u7eb5\u5b50\uff0c\u5176\u8868\u8fbe\u4f1a\u88abDNA\u635f\u4f24\u5242\u663e\u8457\u8bf1\u5bfc\uff0c\u8fd9\u4e9b\u635f\u4f24\u5242\u5305\u62ecDNA\u4ea4\u8054\u5242\u4e1d\u88c2\u9709\u7d20C\uff08MMC\uff09\u3001\u5bfc\u81f4DNA\u65ad\u88c2\u7684\u535a\u6765\u9709\u7d20\u4ee5\u53caDNA\u70f7\u5316\u5242\u7532\u78fa\u9178\u7532\u916f\uff08MMS\uff0924<\/sup>\u3002<\/p>\n\n\n\n

    \u66f4\u91cd\u8981\u7684\u662f\uff0c\u8fd9\u79cd\u8bf1\u5bfc\u4f5c\u7528\u4f9d\u8d56\u4e8e\u7ec6\u83cc\u4e2d\u7ecf\u5178\u7684SOS DNA\u635f\u4f24\u5e94\u7b54\u901a\u8def\u3002\u5b9e\u9a8c\u8868\u660e\uff0c\u8be5\u8fc7\u7a0b\u9700\u8981RecA\u86cb\u767d\u7684\u6fc0\u6d3b\u548cLexA\u963b\u904f\u86cb\u767d\u7684\u5207\u5272\uff0c\u8fd9\u4e24\u8005\u662fSOS\u53cd\u5e94\u7684\u6807\u5fd7\u6027\u4e8b\u4ef6 24<\/sup>\u3002\u8fd9\u4e00\u53d1\u73b0\u76f4\u63a5\u5c06\u7ec6\u80de\u6700\u4e3b\u8981\u7684DNA\u635f\u4f24\u611f\u77e5\u7cfb\u7edf\u4e0e<\/p>\n\n\n\n

    rtc<\/em>\u57fa\u56e0\u7684\u6fc0\u6d3b\u8054\u7cfb\u5728\u4e86\u4e00\u8d77\u3002<\/p>\n\n\n\n

    B. tRNA\u7247\u6bb5\u4f5c\u4e3a\u4e00\u7c7b\u65b0\u578b\u7684\u635f\u4f24\u4fe1\u53f7\u5206\u5b50<\/h3>\n\n\n\n

    \u4e00\u9879\u7a81\u7834\u6027\u7684\u53d1\u73b0\u63ed\u793a\u4e86\u6fc0\u6d3brtc<\/em>\u64cd\u7eb5\u5b50\u7684\u76f4\u63a5\u4fe1\u53f7\u5e76\u975eDNA\u635f\u4f24\u672c\u8eab\uff0c\u800c\u662fDNA\u635f\u4f24\u5e94\u7b54\u8fc7\u7a0b\u4e2d\u79ef\u7d2f\u7684tRNA\u7247\u6bb5 24<\/sup>\u3002\u5728DNA\u635f\u4f24\u540e\uff0c\u7ec6\u80de\u5185\u7684tRNA\u4f1a\u88ab\u7279\u5b9a\u7684\u6838\u9178\u9176\u5207\u5272\uff0c\u4ea7\u751f\u5927\u91cf\u7684tRNA\u7247\u6bb5\u3002<\/p>\n\n\n\n

    \u7814\u7a76\u8868\u660e\uff0c\u8fd9\u4e9btRNA\u7247\u6bb5\uff0c\u7279\u522b\u662f\u672b\u7aef\u5e26\u67092′,3′-\u73af\u78f7\u9178\u76845’\u534a\u4f53\uff0c\u662f\u8f6c\u5f55\u6fc0\u6d3b\u56e0\u5b50RtcR\u7684\u76f4\u63a5\u914d\u4f53\u3002\u5f53tRNA\u7247\u6bb5\u4e0eRtcR\u7684CARF\u7ed3\u6784\u57df\u7ed3\u5408\u540e\uff0c\u4f1a\u4fc3\u4f7fRtcR\u53d1\u751f\u5be1\u805a\u5316\uff0c\u4ece\u800c\u6fc0\u6d3b\u03c354\u4f9d\u8d56\u7684RNA\u805a\u5408\u9176\uff0c\u542f\u52a8rtcBA<\/em>\u64cd\u7eb5\u5b50\u7684\u8f6c\u5f55 24<\/sup>\u3002\u8fd9\u63ed\u793a\u4e86\u4e00\u6761\u7cbe\u5bc6\u7684\u4fe1\u53f7\u4f20\u5bfc\u94fe\uff1aDNA\u635f\u4f24 \u2192 SOS\u53cd\u5e94 \u2192 tRNA\u5207\u5272 \u2192 tRNA\u7247\u6bb5\u4fe1\u53f7 \u2192 RtcR\u6fc0\u6d3b \u2192 RtcA\/B\u8868\u8fbe \u2192 tRNA\u4fee\u590d\u3002<\/p>\n\n\n\n

    C. \u4e00\u4e2a\u4fdd\u5b88\u7684\u5e94\u6fc0\u53cd\u5e94\u89d2\u8272\uff1a\u5728\u80c1\u8feb\u4e0b\u7ef4\u6301\u7ffb\u8bd1\u4fdd\u771f\u5ea6<\/h3>\n\n\n\n

    \u5728E. coli<\/em>\u4e2d\uff0cRtc\u7cfb\u7edf\u7684\u6fc0\u6d3b\u540c\u6837\u4e0e\u7ffb\u8bd1\u673a\u5668\u9762\u4e34\u7684\u6311\u6218\u76f8\u5173\uff0c\u4f8b\u5982\u9776\u5411\u6838\u7cd6\u4f53\u7684\u6297\u751f\u7d20\u6216tRNase\u7684\u4f5c\u7528\u5747\u53ef\u8bf1\u5bfc\u5176\u8868\u8fbe 25<\/sup>\u3002\u7f3a\u4e4fRtc\u7cfb\u7edf\u7684\u7ec6\u80de\u5bf9\u8fd9\u4e9b\u80c1\u8feb\u66f4\u4e3a\u654f\u611f\u3002\u8fd9\u8868\u660e\uff0cRtc\u7cfb\u7edf\u5728\u5e94\u6fc0\u73af\u5883\u4e0b\u7684\u4e3b\u8981\u4f5c\u7528\u662f\u7ef4\u6301\u6838\u7cd6\u4f53\u7684\u7a33\u6001\uff0c\u5e76\u4fee\u590d\u7ffb\u8bd1\u673a\u5668\u53d7\u635f\u7684\u7ec4\u5206\uff08\u5982tRNA\u548crRNA\uff0924<\/sup>\u3002<\/p>\n\n\n\n

    \u7efc\u5408\u6765\u770b\uff0c\u57fa\u56e0\u6bd2\u6027\u5e94\u6fc0\u8bf1\u5bfcRTCB\u901a\u8def\uff0c\u5176\u9996\u8981\u76ee\u7684\u53ef\u80fd\u5e76\u975e\u76f4\u63a5\u4fee\u590dDNA\uff0c\u800c\u662f\u6267\u884c\u4e00\u9879\u81f3\u5173\u91cd\u8981\u7684\u652f\u6301\u6027\u529f\u80fd\uff1a\u7ef4\u62a4\u86cb\u767d\u8d28\u5408\u6210\u673a\u5668\u7684\u5b8c\u6574\u6027\uff0c\u4ee5\u786e\u4fddDNA\u4fee\u590d\u53cd\u5e94\u672c\u8eab\u80fd\u591f\u987a\u5229\u8fdb\u884c<\/strong>\u3002\u4e00\u4e2a\u906d\u53d7DNA\u635f\u4f24\u7684\u7ec6\u80de\u9700\u8981\u5927\u91cf\u5408\u6210DDR\u86cb\u767d\uff08\u5982\u805a\u5408\u9176\u3001\u8fde\u63a5\u9176\u3001\u68c0\u9a8c\u70b9\u86cb\u767d\u7b49\uff09\u3002\u5982\u679c\u4f5c\u4e3a\u7ffb\u8bd1\u539f\u6599\u7684tRNA\u56e0\u9644\u5e26\u635f\u4f24\u800c\u529f\u80fd\u5931\u5e38\uff0c\u6574\u4e2a\u4fee\u590d\u52aa\u529b\u5c06\u529f\u4e8f\u4e00\u7bd1\u3002<\/p>\n\n\n\n

    \u8fd9\u4e2a\u903b\u8f91\u94fe\u6761\u5982\u4e0b\uff1aDNA\u635f\u4f24\u89e6\u53d1\u4e86SOS\u53cd\u5e94\uff0c\u8be5\u53cd\u5e94\u4e0d\u4ec5\u542f\u52a8\u4e86DNA\u4fee\u590d\u57fa\u56e0\u7684\u8868\u8fbe\uff0c\u540c\u65f6\u4e5f\u9884\u89c1\u6216\u68c0\u6d4b\u5230\u4e86\u5bf9tRNA\u7684\u9644\u5e26\u635f\u4f24\u3002\u56e0\u6b64\uff0cSOS\u53cd\u5e94\u201c\u672a\u96e8\u7ef8\u7f2a\u201d\u5730\u6fc0\u6d3b\u4e86Rtc\u7cfb\u7edf\uff0c\u6d3e\u9063\u8fd9\u652f\u201c\u7ffb\u8bd1\u673a\u5668\u62a2\u4fee\u961f\u201d\u53bb\u4fee\u590d\u53d7\u635f\u7684tRNA\uff0c\u786e\u4fddtRNA\u6c60\u7684\u529f\u80fd\u5b8c\u6574\u3002\u901a\u8fc7\u8fd9\u79cd\u65b9\u5f0f\uff0c\u7ec6\u80de\u4fdd\u969c\u4e86DDR\u86cb\u767d\u7684\u201c\u4f9b\u5e94\u94fe\u201d\u7545\u901a\u65e0\u963b\uff0c\u4ece\u800c\u80fd\u591f\u6709\u6548\u5730\u5bf9DNA\u635f\u4f24\u53d1\u8d77\u57fa\u4e8e\u86cb\u767d\u8d28\u7684\u9632\u5fa1\u3002\u56e0\u6b64\uff0cRTCB\u901a\u8fc7\u786e\u4fdd\u4fee\u590d\u86cb\u767d\u7684\u6210\u529f\u5408\u6210\uff0c\u95f4\u63a5\u5730\u4e3a\u57fa\u56e0\u7ec4\u7684\u6210\u529f\u4fee\u590d\u548c\u7a33\u5b9a\u7ef4\u6301\u505a\u51fa\u4e86\u4e0d\u53ef\u6216\u7f3a\u7684\u8d21\u732e\u3002\u8fd9\u6784\u6210\u4e86RTCB\u4e0e\u57fa\u56e0\u7ec4\u7a33\u5b9a\u6027\u4e4b\u95f4\u4e00\u4e2a\u6df1\u523b\u800c\u95f4\u63a5\u7684\u8054\u7cfb\u3002<\/p>\n\n\n\n

    VI. \u751f\u7406\u4e0e\u75c5\u7406\u5b66\u610f\u4e49\uff1aRTCB\u529f\u80fd\u969c\u788d\u3001\u57fa\u56e0\u7ec4\u4e0d\u7a33\u5b9a\u6027\u4e0e\u75be\u75c5<\/h2>\n\n\n\n

    \u5c06\u5206\u5b50\u5c42\u9762\u7684\u53d1\u73b0\u4e0e\u751f\u7269\u4f53\u5065\u5eb7\u8054\u7cfb\u8d77\u6765\uff0c\u53ef\u4ee5\u66f4\u6df1\u523b\u5730\u7406\u89e3RTCB\u7684\u91cd\u8981\u6027\u3002\u6765\u81ea\u54fa\u4e73\u52a8\u7269\u6a21\u578b\u7684\u6570\u636e\u4ee5\u53ca\u4e0e\u4eba\u7c7b\u9057\u4f20\u75c5\u7684\u8868\u578b\u6bd4\u8f83\uff0c\u4e3aRTCB\u5728\u7ef4\u6301\u57fa\u56e0\u7ec4\u7a33\u5b9a\u6027\u4e2d\u7684\u5173\u952e\u4f5c\u7528\u63d0\u4f9b\u4e86\u751f\u7406\u5c42\u9762\u7684\u4f50\u8bc1\u3002<\/p>\n\n\n\n

    A. RTCB\u7f3a\u5931\u5bf9DNA\u4fee\u590d\u57fa\u56e0\u8868\u8fbe\u7684\u5f71\u54cd<\/h3>\n\n\n\n

    \u5728\u5c0f\u9f20\u6a21\u578b\u4e2d\u7684\u7814\u7a76\u63d0\u4f9b\u4e86\u8fc4\u4eca\u4e3a\u6b62RTCB\u4e0e\u54fa\u4e73\u52a8\u7269DDR\u4e4b\u95f4\u6700\u76f4\u63a5\u7684\u8c03\u63a7\u8054\u7cfb\u3002\u5728\u5c0f\u9f20\u5375\u6bcd\u7ec6\u80de\u4e2d\u6761\u4ef6\u6027\u6572\u9664Rtcb<\/em>\u57fa\u56e0\uff0c\u4f1a\u5bfc\u81f4\u5375\u6ce1\u53d1\u80b2\u505c\u6ede\u3001\u5375\u5de2\u65e9\u8870\u548c\u96cc\u6027\u4e0d\u80b2\u7684\u4e25\u91cd\u8868\u578b 26<\/sup>\u3002<\/p>\n\n\n\n

    \u81f3\u5173\u91cd\u8981\u7684\u662f\uff0c\u5bf9\u8fd9\u4e9bRtcb<\/em>\u7f3a\u9677\u578b\u5375\u6bcd\u7ec6\u80de\u7684\u8f6c\u5f55\u7ec4\u5b66\u5206\u6790\u63ed\u793a\uff0cRTCB\u7684\u7f3a\u5931\u5f71\u54cd\u4e86\u4f17\u591a\u57fa\u56e0\u7684\u526a\u63a5\u6a21\u5f0f\u3002\u5176\u4e2d\uff0c\u4e0eDNA\u7532\u57fa\u5316\u548cDNA\u635f\u4f24\u4fee\u590d<\/strong>\u76f8\u5173\u7684\u57fa\u56e0\u8f6c\u5f55\u672c\u7684\u526a\u63a5\u53d7\u5230\u4e86\u663e\u8457\u8c03\u63a7 26<\/sup>\u3002\u8fd9\u4e00\u53d1\u73b0\u610f\u4e49\u91cd\u5927\uff0c\u5b83\u8868\u660eRTCB\u7684RNA\u52a0\u5de5\u6d3b\u6027\u76f4\u63a5\u5f71\u54cd\u7740DDR\u673a\u5668\u7684\u751f\u4ea7\u3002RTCB\u4e0d\u518d\u4ec5\u4ec5\u662f\u4e0eDDR\u86cb\u767d\u7269\u7406\u4e0a\u76f8\u5173\u8054\uff0c\u800c\u662f\u4ecemRNA\u6c34\u5e73\u4e0a\u63a7\u5236\u7740\u5b83\u4eec\u7684\u8868\u8fbe\u548c\u529f\u80fd\u3002<\/p>\n\n\n\n

    B. \u8868\u578b\u91cd\u53e0\uff1aRTCB\u7f3a\u9677\u4e0e\u7ecf\u5178DNA\u4fee\u590d\u7efc\u5408\u5f81\u7684\u6bd4\u8f83<\/h3>\n\n\n\n

    \u8bb8\u591a\u7531DNA\u4fee\u590d\u57fa\u56e0\u7f3a\u9677\u5f15\u8d77\u7684\u4eba\u7c7b\u9057\u4f20\u75c5\uff0c\u5982\u5171\u6d4e\u5931\u8c03\u6bdb\u7ec6\u8840\u7ba1\u6269\u5f20\u75c7\uff08Ataxia-telangiectasia\uff09\u3001\u8303\u53ef\u5c3c\u8d2b\u8840\uff08Fanconi anemia\uff09\u548c\u5e03\u5362\u59c6\u7efc\u5408\u5f81\uff08Bloom syndrome\uff09\uff0c\u5176\u4e34\u5e8a\u7279\u5f81\u5305\u62ec\u57fa\u56e0\u7ec4\u4e0d\u7a33\u5b9a\u6027\u3001\u764c\u75c7\u6613\u611f\u6027\u3001\u65e9\u8870\u4ee5\u53ca\u751f\u80b2\u80fd\u529b\u53d7\u635f\u7b49 16<\/sup>\u3002<\/p>\n\n\n\n

    \u5728Rtcb<\/em>\u6572\u9664\u5c0f\u9f20\u4e2d\u89c2\u5bdf\u5230\u7684\u5375\u5de2\u65e9\u8870\u8868\u578b 26<\/sup>\uff0c\u4e0e\u67d0\u4e9b\u4eba\u7c7bDNA\u4fee\u590d\u7f3a\u9677\u7efc\u5408\u5f81\uff08\u5982BRCA1\u7a81\u53d8\u76f8\u5173\u75be\u75c5\uff09\u7684\u4e34\u5e8a\u8868\u73b0\u5b58\u5728\u91cd\u53e0 28<\/sup>\u3002\u867d\u7136\u8868\u578b\u4e0a\u7684\u76f8\u4f3c\u6027\u4e0d\u80fd\u4f5c\u4e3a\u76f4\u63a5\u8bc1\u636e\uff0c\u4f46\u5b83\u5f3a\u70c8\u6697\u793aRTCB\u901a\u8def\u4e0e\u7ecf\u5178\u7684DDR\u901a\u8def\u5728\u529f\u80fd\u4e0a\u7d27\u5bc6\u4ea4\u7ec7\uff0c\u5171\u540c\u81f4\u529b\u4e8e\u7ef4\u6301\u7ec4\u7ec7\u7684\u957f\u671f\u7a33\u6001\u548c\u57fa\u56e0\u7ec4\u7684\u5b8c\u6574\u6027\u3002<\/p>\n\n\n\n

    \u8fd9\u4e9b\u53d1\u73b0\u5171\u540c\u6307\u5411\u4e00\u4e2a\u66f4\u9ad8\u5c42\u6b21\u7684\u8c03\u63a7\u6a21\u578b\uff1aRTCB\u53ef\u80fd\u4f5c\u4e3a\u57fa\u56e0\u7ec4\u7a33\u5b9a\u6027\u7684\u4e00\u4e2a\u201c\u5143\u8c03\u8282\u5668\u201d\uff08meta-regulator\uff09\uff0c\u901a\u8fc7\u63a7\u5236DDR\u7f51\u7edc\u81ea\u8eab\u7684\u8868\u8fbe\u56fe\u666f\u6765\u53d1\u6325\u4f5c\u7528\u3002\u5176\u89d2\u8272\u8d85\u8d8a\u4e86\u5bf9\u5355\u4e00\u5e95\u7269\uff08\u5982tRNA\u6216XBP1\uff09\u7684\u8fde\u63a5\uff0c\u6269\u5c55\u5230\u5f71\u54cd\u4e00\u7cfb\u5217\u8d1f\u8d23\u7ef4\u62a4\u57fa\u56e0\u7ec4\u7684\u57fa\u56e0\u7684\u526a\u63a5\u6a21\u5f0f\uff0c\u8fdb\u800c\u5f71\u54cd\u5176\u86cb\u767d\u8d28\u4ea7\u7269\u7684\u529f\u80fd\u3002<\/p>\n\n\n\n

    \u8fd9\u4e00\u63a8\u8bba\u7684\u903b\u8f91\u5982\u4e0b\uff1a\u9996\u5148\uff0c\u5728\u5c0f\u9f20\u6a21\u578b\u4e2d\u89c2\u5bdf\u5230RTCB\u7684\u7f3a\u5931\u5bfc\u81f4\u5375\u5de2\u65e9\u8870 26<\/sup>\uff0c\u8fd9\u4e00\u8868\u578b\u4e0e\u67d0\u4e9b\u4eba\u7c7bDNA\u4fee\u590d\u7efc\u5408\u5f81\u76f8\u4f3c 21<\/sup>\u3002\u5176\u6b21\uff0c\u5206\u5b50\u5c42\u9762\u7684\u539f\u56e0\u88ab\u63ed\u793a\u4e3aRTCB\u7f3a\u5931\u5bfc\u81f4\u4e86DNA\u4fee\u590d\u57fa\u56e0\u8f6c\u5f55\u672c\u7684\u526a\u63a5\u7f3a\u9677 26<\/sup>\u3002\u5c06\u8fd9\u4e9b\u89c2\u5bdf\u7ed3\u679c\u7efc\u5408\u8d77\u6765\uff0c\u53ef\u4ee5\u5f97\u51fa\u7ed3\u8bba\uff1aRTCB\u4e0eDDR\u4e4b\u95f4\u7684\u8054\u7cfb\u5e76\u975e\u4ec5\u4ec5\u662f\u76f8\u5173\u6027\uff0c\u800c\u662f\u5b58\u5728\u76f4\u63a5\u7684\u56e0\u679c\u673a\u5236\u3002RTCB\u7684\u7ecf\u5178\u529f\u80fd\u2014\u2014RNA\u8fde\u63a5\/\u526a\u63a5\u2014\u2014\u88ab\u76f4\u63a5\u5e94\u7528\u4e8eDDR\u7cfb\u7edf\u7684mRNA\u4e0a\u3002<\/p>\n\n\n\n

    \u56e0\u6b64\uff0cRTCB\u53ef\u80fd\u662f\u4e00\u4e2a\u4e3b\u5f00\u5173\uff0c\u5e2e\u52a9\u534f\u8c03DNA\u4fee\u590d\u53cd\u5e94\u4e0e\u5176\u4ed6\u7ec6\u80de\u72b6\u6001\u3002\u4f8b\u5982\uff0c\u5728\u9700\u8981\u7ef4\u6301\u6570\u5341\u5e74\u57fa\u56e0\u7ec4\u5b8c\u6574\u6027\u7684\u53d1\u80b2\u4e2d\u7684\u5375\u6bcd\u7ec6\u80de\u4e2d\uff0cRTCB\u53ef\u80fd\u8d1f\u8d23\u751f\u4ea7\u7279\u5b9a\u4e9a\u578b\u7684DDR\u86cb\u767d\uff0c\u4ee5\u9002\u5e94\u957f\u671f\u7ef4\u62a4\u7684\u9700\u6c42\u3002RTCB\u7684\u7f3a\u5931\u7834\u574f\u4e86\u8fd9\u4e00\u7cbe\u7ec6\u8c03\u63a7\u7684\u57fa\u56e0\u8868\u8fbe\u7a0b\u5e8f\uff0c\u5bfc\u81f4\u7ec6\u80de\u51fa\u73b0\u529f\u80fd\u6027\u7684DNA\u4fee\u590d\u7f3a\u9677\u3001\u57fa\u56e0\u7ec4\u4e0d\u7a33\u5b9a\u6027 29<\/sup>\uff0c\u5e76\u6700\u7ec8\u5f15\u53d1\u7ec4\u7ec7\u8870\u7aed\u3002\u8fd9\u4f7fRTCB\u4ece\u4e00\u4e2a\u7b80\u5355\u7684\u8fde\u63a5\u9176\uff0c\u4e0a\u5347\u5230\u4e86DDR\u7f51\u7edc\u7684\u4e0a\u6e38\u8c03\u8282\u8005\uff0c\u5176\u5728\u7ef4\u6301\u751f\u547d\u5065\u5eb7\u4e2d\u7684\u4f5c\u7528\u8fdc\u6bd4\u4e4b\u524d\u6240\u8ba4\u8bc6\u7684\u66f4\u4e3a\u91cd\u8981\u3002<\/p>\n\n\n\n

    VII. \u7efc\u5408\u4e0e\u672a\u6765\u5c55\u671b\uff1aRTCB-DDR\u8f74\u7684\u7edf\u4e00\u6a21\u578b<\/h2>\n\n\n\n

    \u7efc\u5408\u672c\u6587\u63a2\u8ba8\u7684\u5404\u9879\u8bc1\u636e\uff0c\u53ef\u4ee5\u6784\u5efa\u4e00\u4e2a\u5173\u4e8eRTCB\u4e0eDNA\u635f\u4f24\u4fee\u590d\uff08DDR\uff09\u4e4b\u95f4\u5173\u7cfb\u7684\u591a\u5c42\u6b21\u3001\u7efc\u5408\u6027\u6a21\u578b\uff0c\u5e76\u636e\u6b64\u63d0\u51fa\u672a\u6765\u7684\u7814\u7a76\u65b9\u5411\u3002<\/p>\n\n\n\n

    A. \u6574\u5408\u76f4\u63a5\u3001\u95f4\u63a5\u4e0e\u8c03\u63a7\u8bc1\u636e<\/h3>\n\n\n\n

    RTCB\u4e0eDDR\u4e4b\u95f4\u7684\u5173\u7cfb\u5e76\u975e\u5355\u4e00\u7684\u7ebf\u6027\u8054\u7cfb\uff0c\u800c\u662f\u4e00\u4e2a\u5728\u591a\u4e2a\u5c42\u9762\u8fd0\u4f5c\u7684\u590d\u6742\u4e92\u52a8\u7f51\u7edc\uff1a<\/p>\n\n\n\n

      \n
    • \u76f4\u63a5\u7684\u751f\u5316\u8054\u7cfb\uff08\u4e3b\u8981\u57fa\u4e8e\u539f\u6838\u6a21\u578b\uff09<\/strong>\uff1aRTCB\u5bb6\u65cf\u7684\u9176\u53ef\u4ee5\u76f4\u63a5\u5bf9DNA\u672b\u7aef\u8fdb\u884c\u5316\u5b66\u4fee\u9970\uff08\u52a0\u5e3d\uff09\uff0c\u800c\u8fd9\u79cd\u4fee\u9970\u53ef\u4ee5\u88abDDR\u5de5\u5177\u7bb1\u4e2d\u7684\u9176\uff08\u5982Aprataxin\uff09\u8bc6\u522b\u5e76\u5904\u7406\u3002\u8fd9\u63d0\u793a\u4e86\u4e00\u6761\u6f5c\u5728\u7684\u3001\u975e\u7ecf\u5178\u7684DNA\u672b\u7aef\u52a0\u5de5\u901a\u8def\u3002<\/li>\n\n\n\n
    • \u95f4\u63a5\u7684\u7269\u7406\u8054\u7cfb\uff08\u540e\u751f\u52a8\u7269\u6a21\u578b\uff09<\/strong>\uff1a\u5728\u540e\u751f\u52a8\u7269\u4e2d\uff0cRTCB\u4f5c\u4e3atRNA\u8fde\u63a5\u9176\u590d\u5408\u7269\uff08tRNA-LC\uff09\u7684\u4e00\u90e8\u5206\uff0c\u4e0e\u5df2\u77e5\u7684DDR\u86cb\u767dDDX1\u7269\u7406\u8026\u5408\u3002\u8fd9\u8868\u660etRNA-LC\u53ef\u80fd\u662f\u4e00\u4e2a\u529f\u80fd\u6027\u7684\u534f\u8c03\u4e2d\u67a2\uff0c\u7528\u4e8e\u5728RNA\u52a0\u5de5\u548cDNA\u4fee\u590d\u4e4b\u95f4\u4f20\u9012\u4fe1\u53f7\u3002<\/li>\n\n\n\n
    • \u529f\u80fd\u6027\u7684\u8c03\u63a7\u8054\u7cfb\uff08\u7ec6\u83cc\u4e0e\u54fa\u4e73\u52a8\u7269\u6a21\u578b\uff09<\/strong>\uff1a\u5728\u7ec6\u83cc\u4e2d\uff0cRTCB\u901a\u8def\u4f5c\u4e3aSOS\u5e94\u6fc0\u53cd\u5e94\u7684\u4e00\u90e8\u5206\u88ab\u8bf1\u5bfc\uff0c\u4ee5\u4fee\u590d\u53d7\u635f\u7684\u7ffb\u8bd1\u673a\u5668\uff0c\u4ece\u800c\u4fdd\u969cDDR\u86cb\u767d\u7684\u5408\u6210\u3002\u5728\u54fa\u4e73\u52a8\u7269\u4e2d\uff0cRTCB\u901a\u8fc7\u8c03\u63a7\u9009\u62e9\u6027\u526a\u63a5\uff0c\u76f4\u63a5\u5f71\u54cdDDR\u76f8\u5173\u57fa\u56e0\u7684\u8868\u8fbe\uff0c\u626e\u6f14\u7740DDR\u7f51\u7edc\u7684\u4e0a\u6e38\u8c03\u8282\u8005\u89d2\u8272\u3002<\/li>\n<\/ul>\n\n\n\n

      B. \u5173\u952e\u7684\u672a\u89e3\u4e4b\u8c1c\u4e0e\u672a\u6765\u7814\u7a76\u65b9\u5411<\/h3>\n\n\n\n

      \u5c3d\u7ba1\u73b0\u6709\u8bc1\u636e\u5df2\u7ecf\u52fe\u52d2\u51faRTCB-DDR\u8f74\u7684\u8f6e\u5ed3\uff0c\u4f46\u4ecd\u6709\u8bb8\u591a\u5173\u952e\u95ee\u9898\u6709\u5f85\u89e3\u7b54\uff0c\u8fd9\u4e9b\u95ee\u9898\u4e3a\u672a\u6765\u7684\u7814\u7a76\u6307\u660e\u4e86\u65b9\u5411\uff1a<\/p>\n\n\n\n

        \n
      1. RTCB\u7684DNA\u4fee\u9970\u6d3b\u6027\u662f\u5426\u5728\u54fa\u4e73\u52a8\u7269\u7ec6\u80de\u4e2d\u5b58\u5728\uff1f<\/strong> RTCB\u4ecb\u5bfc\u7684DNA\u52a0\u5e3d\u4ee5\u53caRTCB-Aprataxin\u8f74\u662f\u5426\u5728\u54fa\u4e73\u52a8\u7269\u7ec6\u80de\u4e2d\u771f\u5b9e\u5b58\u5728\u5e76\u53d1\u6325\u751f\u7406\u529f\u80fd\uff1f\u8fd9\u662f\u9a8c\u8bc1\u539f\u6838\u6a21\u578b\u662f\u5426\u9002\u7528\u4e8e\u9ad8\u7b49\u751f\u7269\u7684\u5173\u952e\u3002<\/li>\n\n\n\n
      2. tRNA-LC\u5728DNA\u635f\u4f24\u4f4d\u70b9\u7684\u786e\u5207\u4f5c\u7528\u662f\u4ec0\u4e48\uff1f<\/strong> \u6574\u4e2atRNA-LC\u662f\u5426\u4f1a\u88ab\u62db\u52df\u5230DNA\u635f\u4f24\u4f4d\u70b9\uff1f\u5982\u679c\u88ab\u62db\u52df\uff0c\u9664\u4e86DDX1\uff0c\u590d\u5408\u7269\u4e2d\u7684\u5176\u4ed6\u4e9a\u57fa\uff08\u5982RTRAF, FAM98B, Ashwin\uff09\u5728\u8fd9\u4e00\u80cc\u666f\u4e0b\u626e\u6f14\u4f55\u79cd\u89d2\u8272\uff1f<\/li>\n\n\n\n
      3. RTCB\u8c03\u63a7\u7684DDR\u57fa\u56e0\u8c31\u7cfb\u662f\u4ec0\u4e48\uff1f<\/strong> \u5728\u54fa\u4e73\u52a8\u7269\u4e2d\uff0c\u53d7RTCB\u526a\u63a5\u8c03\u63a7\u7684DDR\u76f8\u5173\u8f6c\u5f55\u672c\u7684\u5168\u8c31\u662f\u4ec0\u4e48\uff1f\u8bc6\u522b\u8fd9\u4e9b\u9776\u6807\u5c06\u6709\u52a9\u4e8e\u7cbe\u786e\u63ed\u793aRTCB\u5f71\u54cd\u57fa\u56e0\u7ec4\u7a33\u5b9a\u6027\u7684\u5206\u5b50\u673a\u5236\u3002<\/li>\n\n\n\n
      4. RTCB\u4e0e\u4eba\u7c7b\u75be\u75c5\u7684\u5173\u8054\uff1f<\/strong> \u662f\u5426\u5b58\u5728\u4e0eRTCB\u6216\u5176\u4ed6tRNA-LC\u4e9a\u57fa\u7a81\u53d8\u6216\u8868\u8fbe\u5931\u8c03\u76f8\u5173\u7684\u4eba\u7c7b\u75be\u75c5\uff0c\u7279\u522b\u662f\u764c\u75c7\u6216\u65e9\u8870\u7efc\u5408\u5f81\uff1f<\/li>\n<\/ol>\n\n\n\n

        C. \u6f5c\u5728\u7684\u6cbb\u7597\u610f\u4e49<\/h3>\n\n\n\n

        \u5bf9RTCB-DDR\u8f74\u7684\u6df1\u5165\u7406\u89e3\u4e5f\u53ef\u80fd\u5e26\u6765\u65b0\u7684\u6cbb\u7597\u5951\u673a\u3002\u9274\u4e8eRTCB\u5728\u7ec6\u80de\u5e94\u5bf9\u57fa\u56e0\u6bd2\u6027\u5e94\u6fc0\u4e2d\u7684\u652f\u6301\u4f5c\u7528\uff0cRTCB\u7684\u6291\u5236\u5242\u6709\u6f5c\u529b\u4f5c\u4e3a\u5316\u7597\u6216\u653e\u7597\u7684\u589e\u654f\u5242\u3002\u901a\u8fc7\u6291\u5236RTCB\uff0c\u53ef\u4ee5\u524a\u5f31\u764c\u7ec6\u80de\u4fee\u590d\u5176\u7ffb\u8bd1\u673a\u5668\u7684\u80fd\u529b\uff0c\u4ece\u800c\u963b\u788d\u5176\u6709\u6548\u5e94\u5bf9DNA\u635f\u4f24\u6cbb\u7597\uff0c\u6700\u7ec8\u63d0\u9ad8\u6297\u764c\u7597\u6548\u3002\u53cd\u4e4b\uff0c\u5728\u67d0\u4e9b\u4e0e\u57fa\u56e0\u7ec4\u4e0d\u7a33\u5b9a\u6027\u76f8\u5173\u7684\u65e9\u8870\u6216\u795e\u7ecf\u9000\u884c\u6027\u75be\u75c5\u4e2d\uff0c\u63a2\u7d22\u589e\u5f3aRTCB\u529f\u80fd\u6216\u7a33\u5b9a\u6027\u7684\u7b56\u7565\uff0c\u6216\u8bb8\u80fd\u4e3a\u5ef6\u7f13\u75be\u75c5\u8fdb\u5c55\u63d0\u4f9b\u65b0\u7684\u601d\u8def\u3002\u603b\u800c\u8a00\u4e4b\uff0cRTCB\u5df2\u4ece\u4e00\u4e2a\u5355\u7eaf\u7684RNA\u52a0\u5de5\u9176\uff0c\u8f6c\u53d8\u4e3a\u4e00\u4e2a\u5904\u4e8e\u7ec6\u80de\u7a33\u6001\u7f51\u7edc\u6838\u5fc3\u7684\u5173\u952e\u8c03\u63a7\u8282\u70b9\uff0c\u5176\u4e0eDNA\u635f\u4f24\u4fee\u590d\u7684\u590d\u6742\u4e92\u52a8\u5173\u7cfb\uff0c\u65e0\u7591\u5c06\u6210\u4e3a\u672a\u6765\u751f\u547d\u79d1\u5b66\u7814\u7a76\u7684\u4e00\u4e2a\u524d\u6cbf\u9886\u57df\u3002<\/p>\n","protected":false},"excerpt":{"rendered":"

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