{"id":33543,"date":"2022-07-11T16:27:08","date_gmt":"2022-07-11T07:27:08","guid":{"rendered":"https:\/\/www.hiro-clinic.or.jp\/nipt\/?page_id=33543"},"modified":"2026-03-05T12:19:38","modified_gmt":"2026-03-05T03:19:38","slug":"3-methylcrotonyl-coa-carboxylase-deficiency-1","status":"publish","type":"genetic-diseases","link":"https:\/\/www.hiro-clinic.or.jp\/nipt\/genetic-diseases2\/3-methylcrotonyl-coa-carboxylase-deficiency-1\/","title":{"rendered":"3-\u30e1\u30c1\u30eb\u30af\u30ed\u30c8\u30cb\u30ebCoA\u30ab\u30eb\u30dc\u30ad\u30b7\u30e9\u30fc\u30bc\u6b20\u640d\u75c7"},"content":{"rendered":"\n<h2 class=\"wp-block-heading\">\u6982\u8981<\/h2>\n\n\n\n<p>3-\u30e1\u30c1\u30eb\u30af\u30ed\u30c8\u30cb\u30ebCoA\u30ab\u30eb\u30dc\u30ad\u30b7\u30e9\u30fc\u30bc\u6b20\u640d\u75c7(3-Methylcrotonyl-CoA Carboxylase Deficiency 1)\u306f\u3001\u6f5c\u6027\u907a\u4f1d\u75be\u60a3\u3067\u3042\u308a\u3001\u30ed\u30a4\u30b7\u30f3\u7570\u5316\u904e\u7a0b\u306e\u4e2d\u9593\u4ee3\u8b1d\u7523\u7269\u3067\u3042\u308b3-\u30e1\u30c1\u30eb\u30af\u30ed\u30c8\u30cb\u30eb-CoA\u30923-\u30e1\u30c1\u30eb\u30b0\u30eb\u30bf\u30b3\u30cb\u30eb-CoA\u306b\u5909\u63db\u3059\u308b3-\u30e1\u30c1\u30eb\u30af\u30ed\u30c8\u30cb\u30eb-CoA\u30ab\u30eb\u30dc\u30ad\u30b7\u30e9\u30fc\u30bc(MCC)\u306e\u6b20\u640d\u306b\u3088\u308a\u751f\u3058\u307e\u3059\u3002\u4e73\u5e7c\u5150\u671f\u306b\u611f\u67d3\u75c7\u306e\u7f79\u60a3\u3092\u5951\u6a5f\u306b\u767a\u75c7\u3057\u307e\u3059\u304c\u3001\u7121\u75c7\u72b6\u4f8b\u304b\u3089\u306f\u3001\u91cd\u5ea6\u306e\u767a\u9054\u9045\u6ede\u306a\u3069\u304c\u5831\u544a\u3055\u308c\u3066\u3044\u307e\u3059\u3002<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">\u75ab\u5b66<\/h2>\n\n\n\n<p>\u6b27\u7c73\u3067\u306e\u767a\u75c7\u983b\u5ea6\u306f3.6\uff5e8.5\u4e07\u4eba\u306b1\u4eba\u3067\u3001\u305d\u306e\u3046\u3061\u6709\u75c7\u72b6\u8005\u306f\u5168\u4f53\u306e\u7d0410\uff05\u7a0b\u5ea6\u3001\u3055\u3089\u306b\u91cd\u75c7\u306a\u60a3\u8005\u306f1\uff5e2\uff05\u3068\u8003\u3048\u3089\u308c\u3066\u3044\u307e\u3059\u3002\u672c\u90a6\u3067\u306f\u7d0415\u4e07\u51fa\u751f\u306b1\u4eba\u3068\u63a8\u5b9a\u3055\u308c\u3066\u3044\u307e\u3059\u3002<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">\u539f\u56e0<\/h2>\n\n\n\n<p>\u300cMCC\u300d\u306f\u30df\u30c8\u30b3\u30f3\u30c9\u30ea\u30a2\u306b\u5c40\u5728\u3059\u308b\u9175\u7d20\u3067\u3001\u03b1\u9396\u3068\u03b2\u9396\u306e\u4e8c\u3064\u306e\u7570\u306a\u308b\u30b5\u30d6\u30e6\u30cb\u30c3\u30c8\u304b\u3089\u306a\u308a\u307e\u3059\u3002\u03b1\u9396\u306f\u30d3\u30aa\u30c1\u30f3\u3092\u542b\u6709\u3057\u3066\u304a\u308a\u3001MCCC1(MCCA)\u907a\u4f1d\u5b50\u306b\u30b3\u30fc\u30c9\u3055\u308c\u3001\u03b2\u9396\u306fMCCC2(MCCB)\u907a\u4f1d\u5b50\u306b\u30b3\u30fc\u30c9\u3055\u308c\u3066\u3044\u307e\u3059\u3002\u672c\u75c7\u306f\u03b1\u9396\u3001\u03b2\u9396\u3044\u305a\u308c\u304b\u306e\u6b20\u640d\u306b\u3088\u308a\u751f\u3058\u307e\u3059\u3002<\/p>\n\n\n\u907a\u4f1d\u5b50\u3067\u3042\u308c\u3070\u5f53\u9662\u306e<a href=\"https:\/\/www.hiro-clinic.or.jp\/nipt\/plan\/n-advance\/\">N-advance FM+\u30d7\u30e9\u30f3<\/a>\u30fb<a href=\"https:\/\/www.hiro-clinic.or.jp\/nipt\/plan\/n-advance\/\">N-advance GM+\u30d7\u30e9\u30f3<\/a>\u3067\u691c\u67fb\u304c\u53ef\u80fd\u3068\u306a\u3063\u3066\u304a\u308a\u307e\u3059\u3002\n\n\n<!-- CTA Button -->\n\n<figure class=\"cta-btn\">\n<div class=\"cta-img\">\n<img decoding=\"async\" src=\"\/nipt\/wp-content\/uploads\/2023\/03\/button_pc.png\" alt=\"\u539f\u56e0\">\n<\/div>\n<div class=\"button-1\">\n<img decoding=\"async\" src=\"\/nipt\/wp-content\/uploads\/2023\/03\/cta-btn-orng-2.png\">\n<img decoding=\"async\" class=\"button-2\" src=\"\/nipt\/wp-content\/uploads\/2023\/03\/cta-btn-down.png\" alt=\"blue-btn\">\n<\/div>\n<\/figure>\n\n<style>\n.cta-btn {\n  display: flex;\n  flex-direction: column;\n  align-items: center;\n}\n\n.cta-img {\n  width: 400px;\n  height: auto;\n  padding-top: 3em;\n}\n\n.button-1 {\n  display: flex;\n  flex-direction: column;\n  position: absolute;\n  margin-top: 215px;\n  width: 340px;\n  height: auto;\n}\n\n.button-2 {\n  width: 450px;\n  height: auto;\n}\n<\/style>\n\n\n\n<h2 class=\"wp-block-heading\">\u75c7\u72b6<\/h2>\n\n\n\n<p>\u591a\u304f\u306f\u751f\u5f8c6\u304b\u6708\u304b\u30893\u6b73\u3050\u3089\u3044\u307e\u3067\u306b\u3001\u767a\u71b1\u3084\u4e0b\u75e2\u30fb\u5614\u5410\u306a\u3069\u306e\u6025\u6027\u75be\u60a3\u306b\u7f79\u60a3\u3057\u305f\u969b\u3001\u3051\u3044\u308c\u3093\u3001\u660f\u7761\u3001\u4f4e\u8840\u7cd6\u3001\u55dc\u7720\u3001\u4ee3\u8b1d\u6027\u30a2\u30b7\u30c9\u30fc\u30b7\u30b9\u3001\u9ad8\u30a2\u30f3\u30e2\u30cb\u30a2\u8840\u75c7\u306a\u3069\u306e\u75c7\u72b6\u3067\u767a\u75c7\u3057\u307e\u3059\u3002\u9069\u5207\u306a\u6cbb\u7642\u304c\u3055\u308c\u306a\u3051\u308c\u3070\u3001\u8133\u6d6e\u816b\u304c\u9032\u884c\u3057\u3001\u547d\u304c\u52a9\u304b\u3063\u305f\u3068\u3057\u3066\u3082\u91cd\u5927\u306a\u795e\u7d4c\u5b66\u7684\u5f8c\u907a\u75c7\u3092\u6b8b\u3057\u307e\u3059\u3002\u65b0\u751f\u5150\u30b9\u30af\u30ea\u30fc\u30cb\u30f3\u30b0\u3067\u767a\u898b\u3055\u308c\u305f\u75c7\u4f8b\u306f\u3001\u591a\u304f\u306e\u5834\u5408\u306f\u7121\u75c7\u72b6\u3067\u3059\u304c\u3001\u691c\u67fb\u4e0a\u3001\u4f4e\u30ab\u30eb\u30cb\u30c1\u30f3\u8840\u75c7\u3084\u8efd\u5ea6\u306e\u9ad8\u30a2\u30f3\u30e2\u30cb\u30a2\u8840\u75c7\u3092\u591a\u304f\u5448\u3057\u307e\u3059\u3002\u5bb6\u65cf\u89e3\u6790\u3067\u306f\u3001\u7121\u75c7\u72b6\u306e\u540c\u80de\u4f8b\u304c\u767a\u898b\u3055\u308c\u308b\u3053\u3068\u304c\u3042\u308a\u307e\u3059\u3002<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">\u8a3a\u65ad<\/h2>\n\n\n\n<p>\u6025\u6027\u767a\u4f5c\u6642\uff1a\u4ee3\u8b1d\u6027\u30a2\u30b7\u30c9\u30fc\u30b7\u30b9\u3001\u30b1\u30c8\u30fc\u30b7\u30b9\u3001\u4f4e\u8840\u7cd6\u3001\u9ad8\u30a2\u30f3\u30e2\u30cb\u30a2\u8840\u75c7\u3001\u809d\u969c\u5bb3\u7b49<br>\u5c3f\u4e2d\u6709\u6a5f\u9178\u5206\u6790\uff1a3\u2010\u30d2\u30c9\u30ed\u30ad\u30b7\u30a4\u30bd\u5409\u8349\u9178\u30013\u2010\u30e1\u30c1\u30eb\u30af\u30ed\u30c8\u30cb\u30eb\u30b0\u30ea\u30b7\u30f3\u306e\u6392\u6cc4\u3002<br>\u8840\u4e2d\u30a2\u30b7\u30eb\u30ab\u30eb\u30cb\u30c1\u30f3\u5206\u6790\uff1a\u30d2\u30c9\u30ed\u30ad\u30b7\u30a4\u30bd\u30d0\u30ec\u30ea\u30eb\u30ab\u30eb\u30cb\u30c1\u30f3(C5-OH)\u304c\u4e0a\u6607\u3059\u308b\u305f\u3081\u3001\u30bf\u30f3\u30c7\u30e0\u30de\u30b9\u30b9\u30af\u30ea\u30fc\u30cb\u30f3\u30b0\u306e\u6307\u6a19\u3068\u3057\u3066\u7528\u3044\u3089\u308c\u307e\u3059\u3002\u8840\u4e2d\u904a\u96e2\u30ab\u30eb\u30cb\u30c1\u30f3\u306f\u3057\u3070\u3057\u3070\u4f4e\u4e0b\u3002<br>\u78ba\u5b9a\u8a3a\u65ad\uff1a\u57f9\u990a\u7dda\u7dad\u82bd\u7d30\u80de\u3092\u7528\u3044\u305fMCC\u9175\u7d20\u6d3b\u6027\u6e2c\u5b9a\u3084\u907a\u4f1d\u5b50(MCCC1, MCCC2)\u89e3\u6790\u304c\u6709\u7528\u3002<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">\u6cbb\u7642<\/h2>\n\n\n\n<p>\u6025\u6027\u767a\u4f5c\u6642\u306b\u306f\u3001\u9ad8\u5f35\u7cd6\u6db2\u3092\u7528\u3044\u3066\u7570\u5316\u4f5c\u7528\u3092\u6291\u5236\u3057\u307e\u3059\u3002\u9069\u5b9c\u3001\u30a4\u30f3\u30b9\u30ea\u30f3\u306e\u4f75\u7528\u3084\u30ec\u30dc\u30ab\u30eb\u30cb\u30c1\u30f3\u3001\u30d3\u30bf\u30df\u30f3\u3001\u30a2\u30eb\u30ab\u30ea\u5316\u5264\u306e\u6295\u4e0e\u3092\u884c\u3044\u307e\u3059\u3002 \u6162\u6027\u671f\u306b\u3001\u75c7\u72b6\u306e\u6709\u7121\u306b\u304b\u304b\u308f\u3089\u305a\u4f4e\u30ab\u30eb\u30cb\u30c1\u30f3\u8840\u75c7\u304c\u8a8d\u3081\u3089\u308c\u308b\u5834\u5408\u306f\u3001\u30ec\u30dc\u30ab\u30eb\u30cb\u30c1\u30f3\u3092\u6295\u4e0e\u3057\u307e\u3059\u3002<br>\u4f4e\u86cb\u767d\u98df\u30fb\u30ed\u30a4\u30b7\u30f3\u306e\u5236\u9650\u98df\u304c\u52e7\u3081\u3089\u308c\u3066\u3044\u307e\u3059\u304c\u3001\u305d\u306e\u52b9\u679c\u306f\u4e0d\u660e\u3067\u3059\u3002<\/p>\n\n\n\n<h2 class=\"wp-block-heading\">\u4e88\u5f8c<\/h2>\n\n\n\n<p>\u6025\u6027\u30a2\u30b7\u30c9\u30fc\u30b7\u30b9\u767a\u4f5c\u3092\u8d77\u3053\u3055\u306a\u3051\u308c\u3070\u826f\u597d\u3067\u3059\u3002<\/p>\n\n\n\n<div class=\"wp-block-group references\"><div class=\"wp-block-group__inner-container is-layout-flow wp-block-group-is-layout-flow\">\n<p>\u3010\u53c2\u8003\u6587\u732e\u3011<\/p>\n\n\n\n<ul class=\"wp-block-list\">\n<li>\u5c0f\u5150\u6162\u6027\u7279\u5b9a\u75be\u75c5\u60c5\u5831\u30bb\u30f3\u30bf\u30fc &#8211; <a href=\"https:\/\/www.shouman.jp\/disease\/details\/08_02_027\/\" data-type=\"URL\" data-id=\"https:\/\/www.shouman.jp\/disease\/details\/08_02_027\/\">3-\u30e1\u30c1\u30eb\u30af\u30ed\u30c8\u30cb\u30ebCoA\u30ab\u30eb\u30dc\u30ad\u30b7\u30e9\u30fc\u30bc\u6b20\u640d\u75c7<\/a><\/li>\n\n\n\n<li>\u96e3\u75c5\u60c5\u5831\u30bb\u30f3\u30bf\u30fc &#8211; <a href=\"https:\/\/www.nanbyou.or.jp\/entry\/4825\">\u8907\u5408\u30ab\u30eb\u30dc\u30ad\u30b7\u30e9\u30fc\u30bc\u6b20\u640d\u75c7\uff08\u6307\u5b9a\u96e3\u75c5\uff12\uff15\uff15\uff09<\/a><\/li>\n<\/ul>\n<\/div><\/div>\n","protected":false},"featured_media":0,"menu_order":34,"template":"page_gd.php","class_list":["post-33543","genetic-diseases","type-genetic-diseases","status-publish","hentry"],"acf":[],"_links":{"self":[{"href":"https:\/\/www.hiro-clinic.or.jp\/nipt\/wp-json\/wp\/v2\/genetic-diseases\/33543","targetHints":{"allow":["GET"]}}],"collection":[{"href":"https:\/\/www.hiro-clinic.or.jp\/nipt\/wp-json\/wp\/v2\/genetic-diseases"}],"about":[{"href":"https:\/\/www.hiro-clinic.or.jp\/nipt\/wp-json\/wp\/v2\/types\/genetic-diseases"}],"version-history":[{"count":6,"href":"https:\/\/www.hiro-clinic.or.jp\/nipt\/wp-json\/wp\/v2\/genetic-diseases\/33543\/revisions"}],"predecessor-version":[{"id":125554,"href":"https:\/\/www.hiro-clinic.or.jp\/nipt\/wp-json\/wp\/v2\/genetic-diseases\/33543\/revisions\/125554"}],"wp:attachment":[{"href":"https:\/\/www.hiro-clinic.or.jp\/nipt\/wp-json\/wp\/v2\/media?parent=33543"}],"curies":[{"name":"wp","href":"https:\/\/api.w.org\/{rel}","templated":true}]}}