{"id":414,"date":"2024-10-24T13:06:21","date_gmt":"2024-10-24T04:06:21","guid":{"rendered":"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/?p=414"},"modified":"2024-10-26T14:55:25","modified_gmt":"2024-10-26T05:55:25","slug":"%e5%a4%a7%e9%87%91%e3%81%8c%e5%85%b1%e8%91%97%e3%81%ae%e8%ab%96%e6%96%87%e3%81%8cjci-insight%e3%81%ab%e6%8e%b2%e8%bc%89%e3%81%95%e3%82%8c%e3%81%be%e3%81%97%e3%81%9f","status":"publish","type":"post","link":"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/2024\/10\/24\/%e5%a4%a7%e9%87%91%e3%81%8c%e5%85%b1%e8%91%97%e3%81%ae%e8%ab%96%e6%96%87%e3%81%8cjci-insight%e3%81%ab%e6%8e%b2%e8%bc%89%e3%81%95%e3%82%8c%e3%81%be%e3%81%97%e3%81%9f\/","title":{"rendered":"\u5927\u91d1\u304c\u5171\u8457\u306e\u8ad6\u6587\u304cJCI Insight\u306b\u63b2\u8f09\u3055\u308c\u307e\u3057\u305f"},"content":{"rendered":"<p>\u5927\u91d1\u304c\u5171\u8457\u306b\u5165\u3063\u305f\u8ad6\u6587\u304c\u3001JCI Insight\u306b\u63b2\u8f09\u3055\u308c\u307e\u3057\u305f\u3002\u57fa\u672c\u7684\u306b\u306f\u5171\u540c\u7814\u7a76\u8005\u306e\u7814\u7a76\u3067\u3059\u304c\u3001\u5316\u5408\u7269\u9762\u3067\u5bc4\u4e0e\u3057\u3066\u3044\u308b\u7814\u7a76\u3067\u3059\u3002<\/p>\n<blockquote><p><span class=\"csl-entry\"><span class=\"csl-right-inline\">Azaria, R. D.; Correia, A. B.; Schache, K. J.; Zapata, M.; Pathmasiri, K. C.; Mohanty, V.; Nannapaneni, D. T.; Ashfeld, B. L.; Helquist, P.; Wiest, O.; <span style=\"text-decoration: underline\">Ohgane, K.<\/span>; Li, Q.; Fredenburg, R. A.; Blagg, B. S. J.; Cologna, S. M.; Schultz, M. L.*; Lieberman, A. P.*<br \/>\nMutant Induced Neurons and Humanized Mice Enable Identification of Niemann-Pick C1 Proteostatic Therapies.<br \/>\n<i>JCI Insight <\/i><b>2024<\/b>, <i>9<\/i> (20), e179525. (\u30aa\u30fc\u30d7\u30f3\u30a2\u30af\u30bb\u30b9) <a href=\"https:\/\/insight.jci.org\/articles\/view\/179525\">https:\/\/doi.org\/10.1172\/jci.insight.179525.\u00a0<\/a><\/span><\/span><\/p><\/blockquote>\n<p>\u30cb\u30fc\u30de\u30f3\u30d4\u30c3\u30af\u75c5C\u578b\u306f\u7d30\u80de\u5185\u3078\u306e\u30b3\u30ec\u30b9\u30c6\u30ed\u30fc\u30eb\u306e\u84c4\u7a4d\u3092\u7279\u5fb4\u3068\u3059\u308b\u907a\u4f1d\u6027\u96e3\u75c5\u3067\u3059\u3002\u30ea\u30bd\u30bd\u30fc\u30e0\u306e\u30b3\u30ec\u30b9\u30c6\u30ed\u30fc\u30eb\u30c8\u30e9\u30f3\u30b9\u30dd\u30fc\u30bf\u30fc\u3067\u3042\u308bNPC1\u306b\u5909\u7570\u304c\u5165\u308a\u3001\u6a5f\u80fd\u304c\u5931\u308f\u308c\u308b\u3053\u3068\u3067\u8d77\u3053\u308a\u307e\u3059\u3002\u79c1\u305f\u3061\u306e\u30b0\u30eb\u30fc\u30d7\u3067\u306f\u3001NPC1\u5909\u7570\u4f53\u304c\u30d5\u30a9\u30fc\u30eb\u30c7\u30a3\u30f3\u30b0\u7570\u5e38\u3092\u8d77\u3053\u3057\u3066\u3044\u308b\u3053\u3068\u306b\u7740\u76ee\u3057\u3001NPC1\u5909\u7570\u4f53\u3092\u5b89\u5b9a\u5316\u3057\u3001\u30d5\u30a9\u30fc\u30eb\u30c7\u30a3\u30f3\u30b0\u3092\u52a9\u3051\u308b\u5316\u5408\u7269 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https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/5621deba8a18b839c7a4321764bb05e8-24x9.jpg 24w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/5621deba8a18b839c7a4321764bb05e8-36x13.jpg 36w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/5621deba8a18b839c7a4321764bb05e8-48x17.jpg 48w\" sizes=\"auto, (max-width: 1045px) 100vw, 1045px\" \/><\/p><\/blockquote>\n<p>\u4eca\u56de\u306e\u7814\u7a76\u3067\u306f\u3001\u30d2\u30c8iPS\u7d30\u80de\u304b\u3089\u5206\u5316\u3055\u305b\u305f\u795e\u7d4c\u69d8\u7d30\u80de\u306b\u304a\u3044\u3066\u3001\u3053\u308c\u307e\u3067\u306b\u5831\u544a\u306e\u3042\u308b\u30d7\u30ed\u30c6\u30aa\u30b9\u30bf\u30b7\u30b9\u30ec\u30ae\u30e5\u30ec\u30fc\u30bf\u30fc 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(86%\u306f\u540c\u4e00)\u3001\u30d2\u30c8\u3067\u983b\u5ea6\u306e\u9ad8\u3044I1061T\u5909\u7570\u4f53 (1061\u756a\u76ee\u306e\u30a4\u30bd\u30ed\u30a4\u30b7\u30f3\u304c\u30b9\u30ec\u30aa\u30cb\u30f3\u306b\u5909\u308f\u3063\u305f\u5909\u7570\u4f53)\u306b\u304a\u3044\u3066\u3001\u30d2\u30c8NPC1\u3067\u898b\u3089\u308c\u308b\u30d5\u30a9\u30fc\u30eb\u30c7\u30a3\u30f3\u30b0\u7570\u5e38\u304c\u898b\u3089\u308c\u305a\u3001\u85ac\u7406\u5b66\u7684\u30b7\u30e3\u30da\u30ed\u30f3\u7b49\u306e\u8a55\u4fa1\u304c\u3067\u304d\u306a\u3044\u3068\u3044\u3046\u8ab2\u984c\u304c\u3042\u308a\u307e\u3057\u305f\u3002<\/p>\n<blockquote><p><img loading=\"lazy\" decoding=\"async\" class=\"aligncenter size-full wp-image-416\" src=\"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/2a831ec055ed4fee4e1d0936ea40500d.jpeg\" alt=\"\" width=\"1159\" height=\"548\" srcset=\"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/2a831ec055ed4fee4e1d0936ea40500d.jpeg 1159w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/2a831ec055ed4fee4e1d0936ea40500d-300x142.jpeg 300w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/2a831ec055ed4fee4e1d0936ea40500d-1024x484.jpeg 1024w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/2a831ec055ed4fee4e1d0936ea40500d-768x363.jpeg 768w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/2a831ec055ed4fee4e1d0936ea40500d-24x11.jpeg 24w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/2a831ec055ed4fee4e1d0936ea40500d-36x17.jpeg 36w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/2a831ec055ed4fee4e1d0936ea40500d-48x23.jpeg 48w\" sizes=\"auto, (max-width: 1159px) 100vw, 1159px\" \/><\/p><\/blockquote>\n<p>\u3053\u306e\u7814\u7a76\u3067\u306f\u3001\u30d2\u30c8NPC1\u306eI1061T\u5909\u7570\u4f53\u3092\u6301\u3064\u30e2\u30c7\u30eb\u30de\u30a6\u30b9\u3092\u4f5c\u51fa\u3057\u3001\u30cb\u30fc\u30de\u30f3\u30d4\u30c3\u30af\u75c5C\u578b\u306e\u75c5\u614b\u3092\u518d\u73fe\u3059\u308b\u3053\u3068\u3092\u5831\u544a\u3057\u3066\u3044\u307e\u3059\u3002\u307e\u305f\u3001\u8ad6\u6587\u4e2d\u3067in vivo\u3067\u306e\u52b9\u679c\u306f\u691c\u8a0e\u3055\u308c\u3066\u3044\u307e\u305b\u3093\u304c\u3001\u3053\u306e\u30e2\u30c7\u30eb\u30de\u30a6\u30b9\u7531\u6765\u306e\u7d30\u80de\u306b\u304a\u3044\u3066\u85ac\u7406\u5b66\u7684\u30b7\u30e3\u30da\u30ed\u30f3mo56HC\u304c\u660e\u78ba\u306a\u4f5c\u7528\u3092\u793a\u3059\u3053\u3068\u3092\u5831\u544a\u3057\u3066\u3044\u307e\u3059\u3002<\/p>\n<blockquote><p><img loading=\"lazy\" decoding=\"async\" class=\"aligncenter size-full wp-image-418\" src=\"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/Azaria2024_summary3.jpg\" alt=\"\" width=\"1152\" height=\"748\" srcset=\"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/Azaria2024_summary3.jpg 1152w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/Azaria2024_summary3-300x195.jpg 300w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/Azaria2024_summary3-1024x665.jpg 1024w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/Azaria2024_summary3-768x499.jpg 768w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/Azaria2024_summary3-24x16.jpg 24w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/Azaria2024_summary3-36x23.jpg 36w, https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-content\/uploads\/sites\/31\/2024\/10\/Azaria2024_summary3-48x31.jpg 48w\" sizes=\"auto, (max-width: 1152px) 100vw, 1152px\" \/><\/p><\/blockquote>\n<p>\u57f9\u990a\u7d30\u80de\u30ec\u30d9\u30eb\u3067\u52b9\u679c\u304c\u3042\u308b\u3053\u3068\u3068\u500b\u4f53\u30ec\u30d9\u30eb\u3067\u52b9\u679c\u304c\u3042\u308b\u3053\u3068\u306b\u306f\u5927\u304d\u306a\u9055\u3044\u304c\u3042\u308a\u307e\u3059\u304c\u3001\u500b\u4f53\u30ec\u30d9\u30eb\u3067\u85ac\u7406\u5b66\u7684\u30b7\u30e3\u30da\u30ed\u30f3\u306e\u52b9\u679c\u3092\u691c\u8a3c\u3067\u304d\u308b\u3088\u3046\u306b\u306a\u3063\u305f\u3053\u3068\u3067\u3001\u4eca\u5f8c\u3001\u500b\u4f53\u30ec\u30d9\u30eb\u3067\u4f7f\u3048\u308b\u5316\u5408\u7269\u306e\u63a2\u7d22\u3084\u958b\u767a\u304c\u9032\u3080\u3082\u306e\u3068\u671f\u5f85\u3067\u304d\u307e\u3059\u3002<\/p>\n<p>\u5927\u91d1<\/p>\n","protected":false},"excerpt":{"rendered":"<p>\u5927\u91d1\u304c\u5171\u8457\u306b\u5165\u3063\u305f\u8ad6\u6587\u304c\u3001JCI Insight\u306b\u63b2\u8f09\u3055\u308c\u307e\u3057\u305f\u3002\u57fa\u672c\u7684\u306b\u306f\u5171\u540c\u7814\u7a76\u8005\u306e\u7814\u7a76\u3067\u3059\u304c\u3001\u5316\u5408\u7269\u9762\u3067\u5bc4\u4e0e\u3057\u3066\u3044\u308b\u7814\u7a76\u3067\u3059\u3002 Azaria, R. D.; Correia, A. B.; Schache, K.  &hellip; <a href=\"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/2024\/10\/24\/%e5%a4%a7%e9%87%91%e3%81%8c%e5%85%b1%e8%91%97%e3%81%ae%e8%ab%96%e6%96%87%e3%81%8cjci-insight%e3%81%ab%e6%8e%b2%e8%bc%89%e3%81%95%e3%82%8c%e3%81%be%e3%81%97%e3%81%9f\/\">\u7d9a\u304d\u3092\u8aad\u3080 <span class=\"meta-nav\">&rarr;<\/span><\/a><\/p>\n","protected":false},"author":48,"featured_media":0,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[8],"tags":[],"class_list":["post-414","post","type-post","status-publish","format-standard","hentry","category-ohganelabnews"],"_links":{"self":[{"href":"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-json\/wp\/v2\/posts\/414","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-json\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-json\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-json\/wp\/v2\/users\/48"}],"replies":[{"embeddable":true,"href":"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-json\/wp\/v2\/comments?post=414"}],"version-history":[{"count":5,"href":"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-json\/wp\/v2\/posts\/414\/revisions"}],"predecessor-version":[{"id":424,"href":"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-json\/wp\/v2\/posts\/414\/revisions\/424"}],"wp:attachment":[{"href":"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-json\/wp\/v2\/media?parent=414"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-json\/wp\/v2\/categories?post=414"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/www-p.sci.ocha.ac.jp\/ohganelab\/wp-json\/wp\/v2\/tags?post=414"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}