

{"id":21930,"date":"2015-08-13T00:12:19","date_gmt":"2015-08-12T16:12:19","guid":{"rendered":"https:\/\/case.ntu.edu.tw\/blog\/?p=21930"},"modified":"2018-08-01T16:01:15","modified_gmt":"2018-08-01T08:01:15","slug":"%e3%80%90%e9%86%ab%e5%ad%b8%e7%a0%94%e7%a9%b6%e3%80%91%e7%8b%82%e7%89%9b%e7%97%87%e7%9a%84%e8%a7%a3%e8%97%a5%e5%9c%a8%e6%96%b0%e5%b9%be%e5%85%a7%e4%ba%9e%ef%bc%9f","status":"publish","type":"post","link":"https:\/\/case.ntu.edu.tw\/blog\/?p=21930","title":{"rendered":"\u3010\u91ab\u5b78\u7814\u7a76\u3011\u72c2\u725b\u75c7\u7684\u89e3\u85e5\u5728\u65b0\u5e7e\u5167\u4e9e\uff1f"},"content":{"rendered":"<figure id=\"attachment_21931\" aria-describedby=\"caption-attachment-21931\" style=\"width: 450px\" class=\"wp-caption alignleft\"><img fetchpriority=\"high\" decoding=\"async\" class=\"wp-image-21931\" src=\"http:\/\/case.ntu.edu.tw\/blog\/wp-content\/uploads\/2015\/07\/Histology_bse.jpg\" alt=\"\u72c2\u725b\u75c7\u60a3\u725b\u7684\u8166\u7d44\u7e54\u5207\u7247\u3002\u5716\u7247\u4f86\u6e90\uff1awiki\" width=\"450\" height=\"359\" srcset=\"https:\/\/case.ntu.edu.tw\/blog\/wp-content\/uploads\/2015\/07\/Histology_bse.jpg 700w, https:\/\/case.ntu.edu.tw\/blog\/wp-content\/uploads\/2015\/07\/Histology_bse-600x478.jpg 600w\" sizes=\"(max-width: 450px) 100vw, 450px\" \/><figcaption id=\"caption-attachment-21931\" class=\"wp-caption-text\">\u72c2\u725b\u75c7\u60a3\u725b\u7684\u8166\u7d44\u7e54\u5207\u7247\u3002\u5716\u7247\u4f86\u6e90\uff1awiki<\/figcaption><\/figure>\n<p><strong>\u64b0\u6587\uff5c<\/strong>\u8449\u7da0\u8212<\/p>\n<p>\u72c2\u725b\u75c7(bovine spongiform encephalopathy\uff0cBSE)\u6700\u65e9\u61c9\u8a72\u662f\u5728\u65b0\u5e7e\u5167\u4e9e\u767c\u73fe\u7684\u3002\u7576\u6642\u88ab\u7a31\u70ba\u82e6\u9b6f(kuru)\u75c7\uff0c\u56e0\u70ba\u767c\u75c5\u662f\u7531\u982d\u75db\u958b\u59cb\uff0c\u63a5\u8457\u95dc\u7bc0\u75db\u3001\u56db\u80a2\u986b\u6296\uff08kuru\u662fFore\u8a9e\u4e2d\u7684kuria\/guria\uff0c\u5c31\u662f\u767c\u6296\u7684\u610f\u601d\uff09\u3002\u5b83\u7684\u75c5\u539f\u662f\u666e\u5229\u6602\u86cb\u767d(prion)\uff0c\u9664\u4e86\u9020\u6210\u72c2\u725b\u75c7\u8207\u82e6\u9b6f\u75c7\u4ee5\u5916\uff0c\u9084\u53ef\u4ee5\u5c0e\u81f4\u7f8a\u7684\u6414\u7662\u75c5(scrapie)\u8207\u4eba\u7684CJD(Creutzfeldt\u2013Jakob disease)\u3002<\/p>\n<p>\u5728\u4e8c\u5341\u4e16\u7d00\u4e2d\u671f\uff0c\u5728\u65b0\u5e7e\u5167\u4e9e\u7684\u6fb3\u6d32\u4eba\u767c\u73fe\u4e86\u9019\u500b\u75be\u75c5\u3002\u5f8c\u7e8c\u7684\u7814\u7a76\u767c\u73fe\uff0c\u9019\u500b\u75be\u75c5\u662f\u7531\u65bc\u5403\u4eba\u7684\u7fd2\u4fd7\u6240\u9020\u6210\uff1a\u53ef\u80fd\u57281900\u5e74\u5de6\u53f3\uff0c\u4e00\u4f4dFore\u65cf\u7684\u90e8\u843d\u539f\u4f4f\u6c11\u767c\u751f\u7a81\u8b8a\u7522\u751f\u4e86\u75c5\u539f\uff0c\u7531\u65bc\u4ed6\u5011\u6709\u5403\u4eba\u7684\u98a8\u4fd7\uff0c\u4f7f\u5f97\u9019\u500b\u75be\u75c5\u7531\u4ed6\u958b\u59cb\uff0c\u6f38\u6f38\u50b3\u64ad\u958b\u4f86\u3002\u65bc\u662f\u6b96\u6c11\u5730\u653f\u5e9c\u4fbf\u7981\u6b62\u90e8\u843d\u539f\u4f4f\u6c11\u7e7c\u7e8c\u98df\u4eba\u7684\u7fd2\u4fd7\uff0c\u5c31\u4e0d\u518d\u6709\u65b0\u7684\u75c5\u6b77\u4e86\u3002<!--more--><\/p>\n<p>\u4f46\u662f\uff0c\u7576\u6642\u53c3\u8207\u7814\u7a76\u7684Simon Mead\u6ce8\u610f\u5230\uff0c\u6709\u4e9b\u539f\u4f4f\u6c11\u4f3c\u4e4e\u5c0d\u82e6\u9b6f\u75c7\u6709\u62b5\u6297\u529b\u3002\u7d93\u904e\u7814\u7a76\u4ee5\u5f8c\u767c\u73fe\uff0c\u539f\u4f86\u4ed6\u5011\u7684\u666e\u5229\u6602\u86cb\u767d\u5728\u7b2c127\u500b\u6c28\u57fa\u9178\u767c\u751f\u7a81\u8b8a\uff0c\u7531\u7518\u6c28\u9178\uff08glycine\uff0c\u7b26\u865f\u70baG\uff09\u8b8a\u70ba\u7e88\u6c28\u9178\uff08valine\uff0c\u7b26\u865f\u70baV\uff09\u3002<\/p>\n<p>\u6700\u8fd1\u540c\u4e00\u500b\u7814\u7a76\u5718\u968a\u767c\u8868\u4e86\u91dd\u5c0d\u9019\u500b\u7a81\u8b8a\u9032\u884c\u7684\u9032\u4e00\u6b65\u7684\u7814\u7a76\u3002\u7576\u4ed6\u5011\u5728\u5c0f\u9f20\u9ad4\u5167\u8f49\u5165\u7a81\u8b8a\u7684\u666e\u5229\u6602\u86cb\u767d\uff08\u7c21\u7a31G127V\uff09\u6642\uff0c\u5982\u679c\u5169\u500b\u67d3\u8272\u9ad4\u4e0a\u9762\u7684\u666e\u5229\u6602\u57fa\u56e0\u53ea\u6709\u4e00\u500b\u662fG127V\uff08\u7570\u57fa\u56e0\u578b\uff09\uff0c\u5c0f\u9f20\u5c0d\u82e6\u9b6f\u75c7\u75c5\u539f\u6709\u62b5\u6297\u529b\uff0c\u4f46\u5c0d\u65bcCJD\u75c5\u539f\u7684\u62b5\u6297\u529b\u5247\u8f03\u5f31\u3002<\/p>\n<p>\u4f46\u662f\uff0c\u7576\u5169\u500b\u67d3\u8272\u9ad4\u7684\u57fa\u56e0\u90fd\u662fG127V\u6642\uff0c\u5c0f\u9f20\u5c0d\u7814\u7a76\u5718\u968a\u6e2c\u8a66\u7684\u6240\u6709\u75c5\u539f--\u5305\u62ec\u82e6\u9b6f\u75c7\u3001\u50b3\u7d71\u7684CJD\u4ee5\u53ca\u8b8a\u7570\u578bCJD(vCJD)\u7b49\u2014\u90fd\u5177\u6709\u62b5\u6297\u529b\u3002<\/p>\n<p>\u9019\u500b\u767c\u73fe\u8b93\u7814\u7a76\u5718\u968a\u89ba\u5f97\u5f88\u6709\u610f\u601d\u3002\u70ba\u4ec0\u9ebc\u5462\uff1f\u56e0\u70ba\u57281991\u5e74\u6642\uff0c\u7814\u7a76\u5718\u968a\u66fe\u7d93\u767c\u73fe\uff0c\u5927\u90e8\u5206\u4eba\u7684\u666e\u5229\u6602\u57fa\u56e0\u662f\u7570\u57fa\u56e0\u578b\u7684\uff0c\u800c\u8b8a\u7570\u4e4b\u8655\u5728\u7b2c129\u500b\u6c28\u57fa\u9178\uff0c\u539f\u4f86\u7684\u5e8f\u5217\u61c9\u8a72\u662f\u7532\u786b\u6c28\u9178\uff08methionine\uff0c\u7b26\u865f\u662fM\uff09\u300251%\u7684\u4eba\u662fM129\/V129\uff0c37%\u662fM129\/M129\uff0c\u800c\u5269\u4e0b\u768412%\u662fV129\/V129\u3002\u4f46\u662f\uff0c\u5728\u7f79\u60a3CJD\u7684\u75c5\u4eba\u4e2d\uff0cM129\/V129\u53ea\u4f54\u4e864.5%\u3002\u56e0\u6b64\uff0c\u7814\u7a76\u5718\u968a\u7576\u6642\u7684\u7d50\u8ad6\u662f\uff1a\u666e\u5229\u6602\u86cb\u767d\u8981\u5f62\u6210\u7d50\u6676\u69cb\u9020\u624d\u80fd\u7834\u58de\u8166\u7d44\u7e54\uff0c\u7531\u65bc\u7570\u57fa\u56e0\u578b\u7684\u4eba\u6709\u5169\u7a2e\u666e\u5229\u6602\u86cb\u767d\uff0c\u5e72\u64fe\u4e86\u7d50\u6676\u5f62\u6210\uff0c\u6240\u4ee5\u8f03\u4e0d\u5bb9\u6613\u7f79\u60a3CJD\u3002<\/p>\n<p>\u9019\u4e5f\u662f\u70ba\u4ec0\u9ebc\u7814\u7a76\u5718\u968a\u4e00\u958b\u59cb\u5148\u6e2c\u8a66\u7570\u57fa\u56e0\u578b(V127\/G127)\u7684\u539f\u56e0\uff0c\u4f46\u6700\u5f8c\u537b\u662f\u540c\u57fa\u56e0\u578b\u52dd\u51fa\uff0c\u771f\u7684\u662f\u6709\u9ede\u610f\u5916\u3002<\/p>\n<p>\u4f46\u662f\u66f4\u591a\u610f\u5916\u9084\u5728\u7b49\u8457\u7814\u7a76\u5718\u968a\u3002\u7531\u65bc\u4ed6\u5011\u4f7f\u7528\u7684\u8f49\u57fa\u56e0\u5c0f\u9f20\u6709\u5169\u7a2e\uff0c\u5176\u4e2d\u4e00\u7a2e\u7684G127V\u7684\u8868\u73fe\u91cf\u662f\u91ce\u751f\u7a2e\u5c0f\u9f20\u7684\u5169\u500d\uff0c\u53e6\u4e00\u7a2e\u5247\u8ddf\u91ce\u751f\u7a2e\u76f8\u7576\u3002\u5728\u6e2c\u8a66\u7684\u904e\u7a0b\u4e2d\uff0c\u7814\u7a76\u5718\u968a\u767c\u73fe\uff0c\u82e5\u7570\u57fa\u56e0\u578b\u5c0f\u9f20\u4e2dG127V\u7684\u8868\u73fe\u91cf\u8f03\u9ad8\uff0c\u5247\u5c0d\u65bc\u75c5\u539f\u7684\u62b5\u6297\u529b\u76f8\u5c0d\u8f03\u5f37\u3002<\/p>\n<p>\u9019\u500b\u767c\u73fe\u6709\u4ec0\u9ebc\u610f\u7fa9\u5462\uff1f\u7814\u7a76\u5718\u968a\u8a8d\u70ba\uff0c\u7531\u65bc\u9019\u500b\u7a81\u8b8a\u7684\u666e\u5229\u6602\u86cb\u767d\u4e0d\u50c5\u5c0d\u81f4\u75c5\u7684\u666e\u5229\u6602\u86cb\u767d\u6709\u62b5\u6297\u6027\uff0c\u800c\u4e14\u5927\u91cf\u8868\u73fe\u6642\u53ef\u4ee5\u62b5\u6297\u6240\u6709\u4e0d\u540c\u985e\u578b\u7684\u666e\u5229\u6602\u86cb\u767d\uff1b\u6216\u8a31\u5c07\u4f86\u53ef\u4ee5\u5c07\u9019\u500b\u7a81\u8b8a\u7684\u86cb\u767d\u505a\u6210\u751f\u7269\u88fd\u5291\u4f86\u6cbb\u7642\u5f97\u5230CJD\u7684\u60a3\u8005\u5594\uff01\u00a0\u5982\u6b64\u4e00\u4f86\uff0cCJD\u5c31\u4e0d\u518d\u662f\u7121\u6cd5\u6cbb\u7642\u7684\u75be\u75c5\u4e86\uff01<\/p>\n<p><strong>\u53c3\u8003\u6587\u737b\uff1a<\/strong><br \/>\n1. John Collinge et. al.,1991.Homozygous prion protein genotype predisposes to sporadic Creutzfeldt\u2013Jakob disease. Nature. 352, 340 - 342 ; doi:10.1038\/352340a0<br \/>\n2. John Collinge et. al., 2015. A naturally occurring variant of the human prion protein completely prevents prion disease. Nature. doi:10.1038\/nature14510<\/p>\n<p><span style=\"color: #333399;\"><strong>--<\/strong><\/span><br style=\"color: #000000;\" \/><span style=\"color: #333399;\"><strong>\u4f5c\u8005\uff1a\u8449\u7da0\u8212\u3000\u6148\u6fdf\u5927\u5b78\u751f\u547d\u79d1\u5b78\u7cfb\u52a9\u7406\u6559\u6388\uff0c\u79d1\u6559\u4e2d\u5fc3\u7279\u7d04\u5beb\u624b\uff0c\u5f9e\u4e8b\u79d1\u666e\u6587\u7ae0\u5beb\u4f5c\u3002<\/strong><\/span><\/p>\n<p>&nbsp;<\/p>\n<p><a href=\"https:\/\/line.me\/R\/ti\/p\/%40fcx6182w\" target=\"_blank\" rel=\"noopener noreferrer\"><img decoding=\"async\" class=\"alignnone\" src=\"https:\/\/scdn.line-apps.com\/n\/line_add_friends\/btn\/zh-Hant.png\" alt=\"\u52a0\u5165\u597d\u53cb\" width=\"212\" height=\"36\" border=\"0\"><\/a><\/p>\n","protected":false},"excerpt":{"rendered":"<p>\u64b0\u6587\uff5c\u8449\u7da0\u8212 \u72c2\u725b\u75c7(bovine sp<\/p>\n","protected":false},"author":20,"featured_media":21931,"comment_status":"open","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[3293,1025],"tags":[2004,2006,2005,152,2003,1543,27],"aioseo_notices":[],"jetpack_featured_media_url":"https:\/\/case.ntu.edu.tw\/blog\/wp-content\/uploads\/2015\/07\/Histology_bse.jpg","_links":{"self":[{"href":"https:\/\/case.ntu.edu.tw\/blog\/index.php?rest_route=\/wp\/v2\/posts\/21930"}],"collection":[{"href":"https:\/\/case.ntu.edu.tw\/blog\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/case.ntu.edu.tw\/blog\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/case.ntu.edu.tw\/blog\/index.php?rest_route=\/wp\/v2\/users\/20"}],"replies":[{"embeddable":true,"href":"https:\/\/case.ntu.edu.tw\/blog\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=21930"}],"version-history":[{"count":4,"href":"https:\/\/case.ntu.edu.tw\/blog\/index.php?rest_route=\/wp\/v2\/posts\/21930\/revisions"}],"predecessor-version":[{"id":32312,"href":"https:\/\/case.ntu.edu.tw\/blog\/index.php?rest_route=\/wp\/v2\/posts\/21930\/revisions\/32312"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/case.ntu.edu.tw\/blog\/index.php?rest_route=\/wp\/v2\/media\/21931"}],"wp:attachment":[{"href":"https:\/\/case.ntu.edu.tw\/blog\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=21930"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/case.ntu.edu.tw\/blog\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=21930"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/case.ntu.edu.tw\/blog\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=21930"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}