{"id":1177,"date":"2026-08-31T12:00:00","date_gmt":"2026-08-31T03:00:00","guid":{"rendered":"https:\/\/m.kawasaki-m.ac.jp\/mgm-lyso\/?p=1177"},"modified":"2026-09-26T12:26:02","modified_gmt":"2026-09-26T03:26:02","slug":"%e6%8e%b2%e8%bc%89%e6%83%85%e5%a0%b1%ef%bc%88%e8%ab%96%e6%96%87%ef%bc%89-7","status":"publish","type":"post","link":"https:\/\/m.kawasaki-m.ac.jp\/mgm-lyso\/?p=1177","title":{"rendered":"\u63b2\u8f09\u60c5\u5831\uff08\u8ad6\u6587\uff09"},"content":{"rendered":"\n<p class=\"wp-block-paragraph\"><strong><em>M6PR<\/em>\/<em>IGF2R<\/em>\u4e8c\u91cd\u30ce\u30c3\u30af\u30a2\u30a6\u30c8\u306f\u3001\u30e0\u30b3\u30ea\u30d4\u30c9\u30fc\u30b7\u30b9II\u578b\u304a\u3088\u3073\u30cb\u30fc\u30de\u30f3\u30fb\u30d4\u30c3\u30af\u75c5C2\u578b\u306b\u5bfe\u3059\u308b\u6cbb\u7642\u6cd5\u306e\u958b\u767a\u306b\u3064\u306a\u304c\u308b<\/strong><br><br><br>\u30ea\u30bd\u30bd\u30fc\u30e0\u9175\u7d20\u306eM6P\u5316\u3068\u3001M6P\u30ec\u30bb\u30d7\u30bf\u30fc\u306b\u3088\u308b\u305d\u308c\u3089\u9175\u7d20\u306e\u30ea\u30bd\u30bd\u30fc\u30e0\u3078\u306e\u8f38\u9001\u306f\u30ea\u30bd\u30bd\u30fc\u30e0\u6a5f\u80fd\u306e\u7dad\u6301\u306b\u304a\u3044\u3066\u91cd\u8981\u306a\u30d7\u30ed\u30bb\u30b9\u3067\u3059\u3002\u30ea\u30bd\u30bd\u30fc\u30e0\u9175\u7d20\u306eM6P\u5316\u304c\u969c\u5bb3\u3055\u308c\u308b\u3068\u30e0\u30b3\u30ea\u30d4\u30c9\u30fc\u30b7\u30b9\u3068\u3044\u3046\u75be\u60a3\uff08mucolipidosis II\/III, I-cell disease\uff09\u3092\u767a\u75c7\u3057\u307e\u3059\u3002<br>\u30e0\u30b3\u30ea\u30d4\u30c9\u30fc\u30b7\u30b9\u3067\u306f\u30ea\u30bd\u30bd\u30fc\u30e0\u9175\u7d20\u306eM6P\u5316\u304c\u969c\u5bb3\u3055\u308c\u3066\u3044\u308b\u3082\u306e\u306e\u3001\u305d\u308c\u3092\u8f38\u9001\u3059\u308bM6P\u30ec\u30bb\u30d7\u30bf\u30fc\u7cfb\u306f\u6b63\u5e38\u306b\u4fdd\u305f\u308c\u3066\u3044\u307e\u3059\u3002M6P\u30b7\u30b9\u30c6\u30e0\u306b\u8f38\u9001\u3092\u4f9d\u5b58\u3059\u308b\u30ea\u30bd\u30bd\u30fc\u30e0\u9175\u7d20\u306f\u6570\u5341\u7a2e\u985e\u306b\u6e21\u308b\u305f\u3081\u3001\u6cbb\u7642\u30a2\u30d7\u30ed\u30fc\u30c1\u306b\u304a\u3044\u3066\u5358\u4e00\u306e\u9175\u7d20\u88dc\u5145\u7642\u6cd5\u306e\u7d44\u307f\u5408\u308f\u305b\u306f\u5b9f\u7528\u7684\u3067\u306f\u3042\u308a\u307e\u305b\u3093\u3002\u79c1\u305f\u3061\u306f\u4ee5\u524d\u3001\u6b63\u5e38\u7d30\u80de\u304b\u3089M6P\u5316\u3055\u308c\u305f\u30ea\u30bd\u30bd\u30fc\u30e0\u9175\u7d20\u306e\u6df7\u5408\u7269\u3092\u56de\u53ce\u3057\u3001\u30e0\u30b3\u30ea\u30d4\u30c9\u30fc\u30b7\u30b9\u7d30\u80de\u3078\u6295\u4e0e\u3059\u308b\u3053\u3068\u3067\u7d30\u80de\u6a5f\u80fd\u304c\u56de\u5fa9\u3059\u308b\u3053\u3068\u3092\u78ba\u8a8d\u6e08\u3067\u3059\uff08<a href=\"https:\/\/www.sciencedirect.com\/science\/article\/pii\/S0021925820739098\" target=\"_blank\" rel=\"noopener\"><span style=\"text-decoration: underline;\"><mark style=\"background-color:rgba(0, 0, 0, 0)\" class=\"has-inline-color has-vivid-purple-color\">Otomo T, et al. J Biol Chem 2011<\/mark><\/span><\/a>\uff09\u3002\u305d\u308c\u306b\u3088\u308a\u3001\u30e0\u30b3\u30ea\u30d4\u30c9\u30fc\u30b7\u30b9\u306b\u5bfe\u3059\u308b\u9175\u7d20\u88dc\u5145\u7642\u6cd5\u306e\u30b3\u30f3\u30bb\u30d7\u30c8\u306f\u793a\u3055\u308c\u307e\u3057\u305f\u304c\u3001\u30ea\u30bd\u30bd\u30fc\u30e0\u9175\u7d20\u306e\u6df7\u5408\u7269\u3092\u56de\u53ce\u3059\u308b\u65b9\u6cd5\u306b\u306f\u6539\u5584\u304c\u5fc5\u8981\u3067\u3057\u305f\u3002<br>\u4eca\u56de\u79c1\u305f\u3061\u306f\u3001M6P\u30ec\u30bb\u30d7\u30bf\u30fc\uff082\u7a2e\u985e\uff1a<em>M6PR<\/em>\u3068<em>IGF2R<\/em>\uff09\u3092\u30c0\u30d6\u30eb\u30ce\u30c3\u30af\u30a2\u30a6\u30c8\u3057\u305f\u7d30\u80de\uff08dKO cell\uff09\u3092\u6a39\u7acb\u3057\u307e\u3057\u305f\u3002dKO\u7d30\u80de\u306fM6P\u5316\u306b\u306f\u969c\u5bb3\u306f\u3042\u308a\u307e\u305b\u3093\u304c\u3001\u305d\u306e\u8f38\u9001\u7cfb\u306b\u969c\u5bb3\u304c\u3042\u308b\u305f\u3081\u3001\u30e0\u30b3\u30ea\u30d4\u30c9\u30fc\u30b7\u30b9\u3068\u540c\u69d8\u306b\u7d30\u80de\u5916\u3078\u30ea\u30bd\u30bd\u30fc\u30e0\u9175\u7d20\u3092\u6392\u51fa\u3057\u7d9a\u3051\u307e\u3059\u3002dKO\u7d30\u80de\u304c\u57f9\u990a\u4e0a\u6e05\u4e2d\u306b\u6392\u51fa\u3057\u305f\u30ea\u30bd\u30bd\u30fc\u30e0\u9175\u7d20\u3092\u30e0\u30b3\u30ea\u30d4\u30c9\u30fc\u30b7\u30b9\u7d30\u80de\u3078\u6295\u4e0e\u3059\u308b\u3068\u6a5f\u80fd\u304c\u6539\u5584\u3059\u308b\u3053\u3068\u304c\u78ba\u8a8d\u3067\u304d\u307e\u3057\u305f\u3002\u307e\u305f\u3001\u30ea\u30bd\u30bd\u30fc\u30e0\u9175\u7d20\u306e\u307f\u306a\u3089\u305a\u3001M6P\u7d4c\u8def\u3067\u8f38\u9001\u3055\u308c\u308b\u30ea\u30bd\u30bd\u30fc\u30e0\u30bf\u30f3\u30d1\u30af\u8cea\u306e\u3072\u3068\u3064\u3001NPC2\u30bf\u30f3\u30d1\u30af\u8cea\u3082\u4e0a\u6e05\u4e2d\u306b\u6392\u51fa\u3055\u308c\u3001\u305d\u308c\u3092\u6295\u4e0e\u3055\u308c\u305fNiemann-Pick C2\u7d30\u80de\u306f\u6a5f\u80fd\u3092\u56de\u5fa9\u3057\u307e\u3057\u305f\u3002M6P\u5316\u306e\u969c\u5bb3\u3067\u3042\u308b\u30e0\u30b3\u30ea\u30d4\u30c9\u30fc\u30b7\u30b9\u7d30\u80de\u304c\u7d30\u80de\u5916\u306b\u6392\u51fa\u3059\u308b\u30bf\u30f3\u30d1\u30af\u8cea\u306e\u30d7\u30ed\u30d5\u30a1\u30a4\u30eb\u3068\u3001M6P\u30ec\u30bb\u30d7\u30bf\u30fc\u306e\u969c\u5bb3\u3067\u3042\u308bdKO\u7d30\u80de\u304c\u7d30\u80de\u5916\u306b\u6392\u51fa\u3059\u308b\u30bf\u30f3\u30d1\u30af\u8cea\u306e\u30d7\u30ed\u30d5\u30a1\u30a4\u30eb\u306f\u975e\u5e38\u306b\u985e\u4f3c\u3057\u3066\u304a\u308a\u3001dKO\u7d30\u80de\u306e\u4e0a\u6e05\u306f\u30e0\u30b3\u30ea\u30d4\u30c9\u30fc\u30b7\u30b9\u306b\u304a\u3044\u3066\u6b20\u4e4f\u3057\u3066\u3044\u308b\u30ea\u30bd\u30bd\u30fc\u30e0\u9175\u7d20\u3084\u30bf\u30f3\u30d1\u30af\u8cea\uff08\u7fa4\uff09\u3092\u904e\u4e0d\u8db3\u306a\u304f\u88dc\u5145\u3067\u304d\u308b\u30ea\u30bd\u30fc\u30b9\u3068\u306a\u308a\u3046\u308b\u3068\u8003\u3048\u3089\u308c\u307e\u3059\u3002<br><br><br><strong>M6PR\/IGF2R double-knockout supplies therapeutic sources for mucolipidosis II and Niemann-Pick C2.<\/strong><br>Fujita T, Terawaki S, Moriwaki T, *Otomo T.<br><strong><em>J Biol Chem.<\/em><\/strong> 2026;302(10):113511.<br><a href=\"https:\/\/www.sciencedirect.com\/science\/article\/pii\/S0021925826023835\"><span style=\"text-decoration: underline;\"><mark style=\"background-color:rgba(0, 0, 0, 0)\" class=\"has-inline-color has-vivid-purple-color\">https:\/\/www.sciencedirect.com\/science\/article\/pii\/S0021925826023835<\/mark><\/span><\/a><\/p>\n\n\n\n<p class=\"wp-block-paragraph\"><\/p>\n","protected":false},"excerpt":{"rendered":"<p>M6PR\/IGF2R\u4e8c\u91cd\u30ce\u30c3\u30af\u30a2\u30a6\u30c8\u306f\u3001\u30e0\u30b3\u30ea\u30d4\u30c9\u30fc\u30b7\u30b9II\u578b\u304a\u3088\u3073\u30cb\u30fc\u30de\u30f3\u30fb\u30d4\u30c3\u30af\u75c5C2\u578b\u306b\u5bfe\u3059\u308b\u6cbb\u7642\u6cd5\u306e\u958b\u767a\u306b\u3064\u306a\u304c\u308b \u30ea\u30bd\u30bd\u30fc\u30e0\u9175\u7d20\u306eM6P\u5316\u3068\u3001M6P\u30ec\u30bb\u30d7\u30bf\u30fc\u306b\u3088\u308b\u305d\u308c\u3089\u9175\u7d20\u306e\u30ea\u30bd\u30bd\u30fc\u30e0\u3078\u306e\u8f38\u9001\u306f\u30ea\u30bd\u30bd\u30fc\u30e0\u6a5f\u80fd&hellip;<\/p>\n","protected":false},"author":1,"featured_media":0,"comment_status":"closed","ping_status":"closed","sticky":false,"template":"","format":"standard","meta":{"footnotes":""},"categories":[3],"tags":[],"class_list":["post-1177","post","type-post","status-publish","format-standard","hentry","category-3"],"_links":{"self":[{"href":"https:\/\/m.kawasaki-m.ac.jp\/mgm-lyso\/index.php?rest_route=\/wp\/v2\/posts\/1177","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/m.kawasaki-m.ac.jp\/mgm-lyso\/index.php?rest_route=\/wp\/v2\/posts"}],"about":[{"href":"https:\/\/m.kawasaki-m.ac.jp\/mgm-lyso\/index.php?rest_route=\/wp\/v2\/types\/post"}],"author":[{"embeddable":true,"href":"https:\/\/m.kawasaki-m.ac.jp\/mgm-lyso\/index.php?rest_route=\/wp\/v2\/users\/1"}],"replies":[{"embeddable":true,"href":"https:\/\/m.kawasaki-m.ac.jp\/mgm-lyso\/index.php?rest_route=%2Fwp%2Fv2%2Fcomments&post=1177"}],"version-history":[{"count":4,"href":"https:\/\/m.kawasaki-m.ac.jp\/mgm-lyso\/index.php?rest_route=\/wp\/v2\/posts\/1177\/revisions"}],"predecessor-version":[{"id":1181,"href":"https:\/\/m.kawasaki-m.ac.jp\/mgm-lyso\/index.php?rest_route=\/wp\/v2\/posts\/1177\/revisions\/1181"}],"wp:attachment":[{"href":"https:\/\/m.kawasaki-m.ac.jp\/mgm-lyso\/index.php?rest_route=%2Fwp%2Fv2%2Fmedia&parent=1177"}],"wp:term":[{"taxonomy":"category","embeddable":true,"href":"https:\/\/m.kawasaki-m.ac.jp\/mgm-lyso\/index.php?rest_route=%2Fwp%2Fv2%2Fcategories&post=1177"},{"taxonomy":"post_tag","embeddable":true,"href":"https:\/\/m.kawasaki-m.ac.jp\/mgm-lyso\/index.php?rest_route=%2Fwp%2Fv2%2Ftags&post=1177"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}