{"id":646,"date":"2017-03-14T11:50:30","date_gmt":"2017-03-14T02:50:30","guid":{"rendered":"http:\/\/www2.med.osaka-u.ac.jp\/wwwmed\/eng\/activities\/results\/2007year\/murakami-masaaki"},"modified":"2017-04-28T15:30:07","modified_gmt":"2017-04-28T06:30:07","slug":"article02","status":"publish","type":"page","link":"https:\/\/www.med.osaka-u.ac.jp\/eng\/activities\/results\/2007year\/article02","title":{"rendered":"MURAKAMI Masaaki \u226aDevelopmental Immunology\u226b <span>\u201cHomeostatic proliferating CD4 T cells are involved in the pathogenesis of an Omenn syndrome murine model\u201d<\/span>"},"content":{"rendered":"<ul class=\"linkBar clearfix\">\n<li> <a href=\"http:\/\/www.med.osaka-u.ac.jp\/activities\/results\/2007year\/article02\">Text in Japanese<\/a><\/li>\n<\/ul>\n<p><!-- \n\n<h1>\u6751\u4e0a \u6b63\u6643 \u226a\u514d\u75ab\u767a\u751f\u5b66\u226b<\/h1>\n\n --><\/p>\n<p class=\"figure\"><a href=\"http:\/\/www.med.osaka-u.ac.jp\/eng\/wp-content\/uploads\/\/activities\/results\/2007\/hirano20070501.jpeg\" target=\"_blank\" class=\"zoom\"><img loading=\"lazy\" decoding=\"async\" src=\"http:\/\/www.med.osaka-u.ac.jp\/eng\/wp-content\/uploads\/\/activities\/results\/2007\/hirano20070501.jpeg\" alt=\"\" height=\"158\" width=\"200\" \/><br \/>\n  <span class=\"click\">Click to enlarge<\/span><\/a><\/p>\n<p>2007-5-1<br \/>\n  <span class=\"lineFrame\">Publish<\/span> J. Clin. Invest. 117: 1270-1281, 2007<\/p>\n<p><!-- \n\n<h3>CD4+ T\u7d30\u80de\u306e\u30db\u30e1\u30aa\u30b9\u30bf\u30b7\u30b9\u5897\u6b96\u306f\u30de\u30a6\u30b9\u306e\u30aa\u30fc\u30e1\u30f3\u75c7\u5019\u7fa4\u30e2\u30c7\u30eb\u306e\u767a\u75c7\u539f\u56e0\u306b\u95a2\u4e0e\u3059\u308b<\/h3>\n\n\n\n\n<p>\u8981\u65e8\uff1a\u30aa\u30fc\u30e1\u30f3\u75c7\u5019\u7fa4\u306e\u60a3\u8005\u306b\u306fRAG\u907a\u4f1d\u5b50\u306e\u6a5f\u80fd\u3092\u5f31\u3081\u308b\u5909\u7570\u304c\u3042\u308a\u3001\u3055\u307e\u3056\u307e\u306a\u91cd\u75c7\u8907\u5408\u578b\u514d\u75ab\u4e0d\u5168\u3092\u767a\u75c7  \u3059\u308b\u3002\u3053\u306e\u3046\u3061\u3001\u3069\u306e\u75c7\u72b6\u304cRAG\u907a\u4f1d\u5b50\u306e\u5909\u7570\u306b\u3088\u308b\u76f4\u63a5\u7684\u306a\u3082\u306e\u3067\u3001\u3069\u308c\u304c\u4ed6\u306e\u907a\u4f1d\u591a\u578b\u306b\u4f9d\u5b58\u3057\u3066\u3044\u308b\u306e\u304b\u306f\u77e5\u3089\u308c\u3066\u3044\u306a\u304b\u3063\u305f\u3002\u3053\u308c\u3092\u7406\u89e3\u3059\u308b\u305f\u3081\u306b  \u306f\u30aa\u30fc\u30e1\u30f3\u75c7\u5019\u7fa4\u306e\u52d5\u7269\u30e2\u30c7\u30eb\u304c\u5fc5\u8981\u3067\u3042\u3063\u305f\u3002\u6211\u3005\u306f\u3001RAG1\u907a\u4f1d\u5b50\u306b\u5909\u7570\u3092\u6301\u3061\u3001\u305d\u306e\u6d3b\u6027\u304c\u6e1b\u5f31\u3057\u3066\u3044\u308bC57BL\/10\u7a81\u7136\u5909\u7570\u30de\u30a6\u30b9\u3092\u767a\u898b\u3057\u305f\u3002  \u305d\u306e\u500b\u4f53\u306e\u5b50\u5b6b\u3067\u306f\u3001\u30e1\u30e2\u30ea\u30fc\u8868\u73fe\u578bT\u7d30\u80de\u304c\u5897\u52a0\u3057\u3001\u809d\u81d3\u3068\u813e\u81d3\u306b\u816b\u5927\u3068\u597d\u9178\u7403\u6d78\u6f64\u304c\u8a8d\u3081\u3089\u308c\u3001T\u7d30\u80de\u306f\u30aa\u30ea\u30b4\u30af\u30ed\u30fc\u30ca\u30eb\u3067\u3042\u308a\u3001\u305d\u3057\u3066IgE\u304c\u9ad8\u3044\u3068  \u3044\u3063\u305f\u30aa\u30fc\u30e1\u30f3\u75c7\u5019\u7fa4\u306e\u4e3b\u306a\u75c7\u72b6\u304c\u898b\u3089\u308c\u305f\u3002CD4+   T\u7d30\u80de\u3092\u9664\u53bb\u3059\u308b\u3053\u3068\u306b\u3088\u3063\u3066\u3001\u9ad8IgE\u75c7\u304c\u6539\u5584\u3057\u305f\u3002\u5f93\u3063\u3066\u3001\u3053\u306e\u300c\u30e1\u30e2\u30ea\u30fc\u5909\u7570\u4f53(MM)\u300d\u30de\u30a6\u30b9\u306f\u30d2\u30c8\u306e\u30aa\u30fc\u30e1\u30f3\u75c7\u5019\u7fa4\u306e\u52d5\u7269\u30e2\u30c7\u30eb\u3067\u3042\u308a\u3001\u30aa\u30fc\u30e1  \u30f3\u75c7\u5019\u7fa4\u306e\u591a\u304f\u306e\u75c7\u72b6\u304cRAG\u907a\u4f1d\u5b50\u306e\u5909\u7570\u306b\u3088\u3063\u3066\u5f15\u304d\u8d77\u3053\u3055\u308c\u3001\u307e\u305f\u3044\u304f\u3064\u304b\u306e\u75c7\u72b6\u306fCD4+ T\u7d30\u80de\u306e\u7570\u5e38\u304c\u539f\u56e0\u3067\u3042\u308b\u3053\u3068\u3092\u660e\u3089\u304b\u3068\u306a\u3063\u305f\u3002<\/p>\n\n --><\/p>\n<p> Patients with Omenn syndrome (OS) have hypomorphic RAG   mutations and develop varying manifestations of severe combined   immunodeficiency. It is not known which symptoms are caused directly by   the RAG mutations and which depend on other polymorphic genes. Our   current understanding of OS is limited by the lack of an animal model.   In the present study, we identified a C57BL\/10 mouse with a spontaneous   mutation in, and reduced activity of, RAG1. Mice bred from this animal   contained high numbers of memory-phenotype T cells and experienced   hepatosplenomegaly and eosinophilia, had oligoclonal T cells, and   demonstrated elevated levels of IgE, major symptoms of OS. Depletion of   CD4+ T cells in the mice caused a reduction in their IgE levels. Hence   these &quot;memory mutant&quot; mice are a model for human OS; many symptoms of   their disease were direct results of the Rag hypomorphism and some were   caused by malfunctions of their CD4+ T cells.<\/p>\n<p class=\"mt25\"><span class=\"lineFrame\">URL<\/span> <a href=\"http:\/\/www.med.osaka-u.ac.jp\/pub\/molonc\/www\/\" target=\"_blank\">http:\/\/www.med.osaka-u.ac.jp\/pub\/molonc\/www\/<\/a><\/p>\n","protected":false},"excerpt":{"rendered":"<p>Text in Japanese Click to enlarge 2007-5-1 Publish J. Clin. Invest. 117: 1270-1281, 2007 Patients with Omenn s [&hellip;]<\/p>\n","protected":false},"author":1,"featured_media":1816,"parent":464,"menu_order":166,"comment_status":"closed","ping_status":"closed","template":"","meta":{"footnotes":""},"_links":{"self":[{"href":"https:\/\/www.med.osaka-u.ac.jp\/eng\/wp-json\/wp\/v2\/pages\/646"}],"collection":[{"href":"https:\/\/www.med.osaka-u.ac.jp\/eng\/wp-json\/wp\/v2\/pages"}],"about":[{"href":"https:\/\/www.med.osaka-u.ac.jp\/eng\/wp-json\/wp\/v2\/types\/page"}],"author":[{"embeddable":true,"href":"https:\/\/www.med.osaka-u.ac.jp\/eng\/wp-json\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/www.med.osaka-u.ac.jp\/eng\/wp-json\/wp\/v2\/comments?post=646"}],"version-history":[{"count":6,"href":"https:\/\/www.med.osaka-u.ac.jp\/eng\/wp-json\/wp\/v2\/pages\/646\/revisions"}],"predecessor-version":[{"id":2365,"href":"https:\/\/www.med.osaka-u.ac.jp\/eng\/wp-json\/wp\/v2\/pages\/646\/revisions\/2365"}],"up":[{"embeddable":true,"href":"https:\/\/www.med.osaka-u.ac.jp\/eng\/wp-json\/wp\/v2\/pages\/464"}],"wp:featuredmedia":[{"embeddable":true,"href":"https:\/\/www.med.osaka-u.ac.jp\/eng\/wp-json\/wp\/v2\/media\/1816"}],"wp:attachment":[{"href":"https:\/\/www.med.osaka-u.ac.jp\/eng\/wp-json\/wp\/v2\/media?parent=646"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}