[{"@context":"http:\/\/schema.org\/","@type":"BlogPosting","@id":"https:\/\/wiki.edu.vn\/jp\/wiki9\/archives\/108320#BlogPosting","mainEntityOfPage":"https:\/\/wiki.edu.vn\/jp\/wiki9\/archives\/108320","headline":"\u7b2cVIII\u56e0\u5b50 – Wikipedia","name":"\u7b2cVIII\u56e0\u5b50 – Wikipedia","description":"F8 PDB\u306b\u767b\u9332\u3055\u308c\u3066\u3044\u308b\u69cb\u9020 PDB \u30aa\u30eb\u30bd\u30ed\u30b0\u691c\u7d22: RCSB PDBe PDBj PDB\u306eID\u30b3\u30fc\u30c9\u4e00\u89a7 1CFG, 1D7P, 1IQD, 2R7E, 3CDZ, 3HNB, 3HNY, 3HOB, 4BDV, 1FAC, 3J2Q, 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RCSB PDBe PDBj PDB\u306eID\u30b3\u30fc\u30c9\u4e00\u89a71CFG, 1D7P, 1IQD, 2R7E, 3CDZ, 3HNB, 3HNY, 3HOB, 4BDV, 1FAC, 3J2Q, 3J2S, 4KI5, 4PT6, 4XZU\u8b58\u5225\u5b50\u8a18\u53f7F8, AHF, DXS1253E, F8B, F8C, FVIII, HEMA, coagulation factor VIII\u5916\u90e8IDOMIM: 300841 MGI: 88383 HomoloGene: 49153 GeneCards: F8 \u30aa\u30eb\u30bd\u30ed\u30b0\u7a2e\u30d2\u30c8\u30de\u30a6\u30b9EntrezEnsemblUniProtRefSeq(mRNA)RefSeq(\u30bf\u30f3\u30d1\u30af\u8cea)\u5834\u6240(UCSC)Chr X: 154.84 \u2013 155.03 MbChr X: 74.22 \u2013 74.43 MbPubMed\u691c\u7d22[3][4]\u30a6\u30a3\u30ad\u30c7\u30fc\u30bf \u7b2cVIII\u56e0\u5b50\uff08\u3060\u30448\u3044\u3093\u3057\u3001Factor VIII\u3001FVIII\uff09\u3068\u306f\u3001\u8840\u6db2\u51dd\u56fa\u306b\u5fc5\u9808\u306e\u30bf\u30f3\u30d1\u30af\u8cea\u306e1\u3064\u3067\u3042\u308b\u3002\u8840\u6db2\u51dd\u56fa\u30ab\u30b9\u30b1\u30fc\u30c9\u3092\u69cb\u6210\u3059\u308b\u30bf\u30f3\u30d1\u30af\u8cea\u306e1\u3064\u3067\u3042\u308a\u3001\u5065\u5e38\u306a\u30d2\u30c8\u306e\u8840\u6f3f\u306b\u306f\u5fc5\u305a\u542b\u307e\u308c\u3066\u304a\u308a\u3001\u5e38\u306b\u5168\u8eab\u306e\u8840\u7ba1\u5185\u3092\u5de1\u3063\u3066\u3044\u308b\u3002\u7b2cVIII\u56e0\u5b50\u3092\u30b3\u30fc\u30c9\u3057\u3066\u3044\u308b\u907a\u4f1d\u5b50\u306b\u7570\u5e38\u3092\u6301\u3061\u3001\u3053\u308c\u3092\u4e0a\u624b\u304f\u4f5c\u308c\u306a\u3044\u3068\u8840\u6db2\u51dd\u56fa\u304c\u6b63\u5e38\u306b\u884c\u308f\u308c\u305a\u3001\u8840\u53cb\u75c5A\u3092\u767a\u75c7\u3059\u308b\u3002\u306a\u304a\u3001\u7b2cVIII\u56e0\u5b50\u304c\u6d3b\u6027\u5316\u3055\u308c\u305f\u72b6\u614b\uff08activation\uff09\u306f\u3001\u7b2cVIIIa\u56e0\u5b50\u3068\u547c\u3070\u308c\u308b\u3002\u30d2\u30c8\u306e\u5834\u5408\u3001\u7b2cVIII\u56e0\u5b50\u306f\u809d\u81d3\u306e\u985e\u6d1e\u5185\u76ae\u7d30\u80de\u3068\u3001\u809d\u81d3\u4ee5\u5916\u306e\u4f53\u4e2d\u306e\u5185\u76ae\u7d30\u80de\u3067\u7523\u751f\u3055\u308c\u308b\u3002\u8840\u7ba1\u3092\u640d\u50b7\u3059\u308b\u50b7\u5bb3\u304c\u8d77\u3053\u308b\u307e\u3067\u3001\u7b2cVIII\u56e0\u5b50\u306fvon Willebrand\u56e0\u5b50\u3068\u547c\u3070\u308c\u308b\u5225\u306e\u5206\u5b50\u3068\u7d50\u5408\u3057\u305f\u4e0d\u6d3b\u6027\u72b6\u614b\u3067\u8840\u6d41\u4e2d\u3092\u5faa\u74b0\u3059\u308b[5]\u3002\u50b7\u5bb3\u306b\u53cd\u5fdc\u3057\u3066\u7b2cVIII\u56e0\u5b50\u306f\u6d3b\u6027\u5316\u3055\u308c\u3001von Willebrand\u56e0\u5b50\u304b\u3089\u5206\u96e2\u3055\u308c\u308b\u3002\u7b2cVIII\u56e0\u5b50\u306e\u6d3b\u6027\u578b\u30bf\u30f3\u30d1\u30af\u8cea\u306f\u7b2cVIIIa\u56e0\u5b50\u3068\u547c\u3070\u308c\u3001\u3053\u308c\u306f\u7b2cIX\u56e0\u5b50\u3068\u547c\u3070\u308c\u308b\u5225\u306e\u51dd\u56fa\u56e0\u5b50\u3068\u76f8\u4e92\u4f5c\u7528\u3059\u308b\u3002\u3053\u306e\u76f8\u4e92\u4f5c\u7528\u304c\u3001\u8840\u6813\u3092\u5f62\u6210\u3059\u308b\u5225\u306e\u5316\u5b66\u53cd\u5fdc\u306e\u9023\u9396\u3092\u958b\u59cb\u3055\u305b\u308b[5]\u3002\u7b2cVIIIa\u56e0\u5b50\u306f\u7b2cIXa\u56e0\u5b50\u306e\u88dc\u56e0\u5b50\u3067\u3042\u308a\u3001\u30ab\u30eb\u30b7\u30a6\u30e0\u30a4\u30aa\u30f3\u3068\u30ea\u30f3\u8102\u8cea\u306e\u5b58\u5728\u4e0b\u3067\u8907\u5408\u4f53\u3092\u5f62\u6210\u3057\u3001\u3053\u306e\u8907\u5408\u4f53\u306f\u7b2cX\u56e0\u5b50\u3092\u6d3b\u6027\u578b\u3067\u3042\u308bXa\u56e0\u5b50\u3078\u3068\u5909\u63db\u3059\u308b\u306e\u3067\u3042\u308b\u3002\u30d2\u30c8\u306e\u7b2cVIII\u56e0\u5b50\u306f\u3001X\u67d3\u8272\u4f53\u306b\u5185\u306eF8\u907a\u4f1d\u5b50\u306b\u30b3\u30fc\u30c9\u3055\u308c\u3066\u3044\u308b[6][7]\u3002\u7b2cVIII\u56e0\u5b50\u306e\u907a\u4f1d\u5b50\u306f\u3001\u9078\u629e\u7684\u30b9\u30d7\u30e9\u30a4\u30b7\u30f3\u30b0\u306b\u3088\u3063\u30662\u7a2e\u985e\u306e\u8ee2\u5199\u7523\u7269\u3092\u7523\u751f\u3059\u308b\u3002\u8ee2\u5199\u30d0\u30ea\u30a2\u30f3\u30c81\u306f\u5de8\u5927\u306a\u7cd6\u30bf\u30f3\u30d1\u30af\u8cea\u3067\u3042\u308b\u30a2\u30a4\u30bd\u30d5\u30a9\u30fc\u30e0a\u3092\u30b3\u30fc\u30c9\u3057\u3001von Willebrand\u56e0\u5b50\u3068\u975e\u5171\u6709\u7d50\u5408\u306b\u3088\u3063\u3066\u8907\u5408\u4f53\u3092\u5f62\u6210\u3057\u305f\u72b6\u614b\u3067\u8840\u6f3f\u4e2d\u306b\u542b\u307e\u308c\u3001\u5168\u8eab\u306e\u8840\u7ba1\u5185\u3092\u4e0d\u6d3b\u6027\u306a\u72b6\u614b\u306e\u307e\u307e\u3067\u5faa\u74b0\u3059\u308b\u3002\u305d\u306e\u5f8c\u3001\u3053\u306e\u30bf\u30f3\u30d1\u30af\u8cea\u306f\u3001\u5fc5\u8981\u306b\u5fdc\u3058\u3066\u8907\u6570\u56de\u306e\u5207\u65ad\u304c\u884c\u308f\u308c\u308b\u3002\u8ee2\u5199\u30d0\u30ea\u30a2\u30f3\u30c82\u306f\u5c0f\u3055\u306a\u30bf\u30f3\u30d1\u30af\u8cea\u3067\u3042\u308b\u30a2\u30a4\u30bd\u30d5\u30a9\u30fc\u30e0b\u3092\u30b3\u30fc\u30c9\u3057\u3066\u3044\u308b\u3068\u63a8\u5b9a\u3055\u308c\u3001\u7b2cVIIIc\u56e0\u5b50\u306e\u30ea\u30f3\u8102\u8cea\u7d50\u5408\u30c9\u30e1\u30a4\u30f3\u3092\u69cb\u6210\u3059\u308b\u3002\u3053\u306e\u7d50\u5408\u30c9\u30e1\u30a4\u30f3\u306f\u8840\u6db2\u51dd\u56fa\u6d3b\u6027\u306b\u5fc5\u9808\u3067\u3042\u308b[8]\u3002\u7b2cVIII\u56e0\u5b50\u306e\u907a\u4f1d\u5b50\u306e\u6b20\u9665\u306f\u3001X\u9023\u9396\u52a3\u6027\u907a\u4f1d\u306e\u8840\u6db2\u51dd\u56fa\u969c\u5bb3\u3067\u3042\u308b\u8840\u53cb\u75c5A\u3092\u5f15\u304d\u8d77\u3053\u3059[9]\u3002\u3053\u306e\u305f\u3081\u7b2cVIII\u56e0\u5b50\u306f\u3001\u6297\u8840\u53cb\u75c5\u56e0\u5b50A (anti-hemophilic factor A, AHFA) \u3068\u3057\u3066\u3082\u77e5\u3089\u308c\u308b[\u6ce8\u91c8 1]\u3002\u8840\u53cb\u75c5A\u306e\u5bfe\u75c7\u7642\u6cd5\u3068\u3057\u3066\u3001\u7b2cVIII\u56e0\u5b50\u88fd\u5264\uff08\u82f1\u8a9e\u7248\uff09\u304c\u5fc5\u8981\u3068\u3055\u308c\u3066\u3044\u308b\u3002\u3053\u306e\u7b2cVIII\u56e0\u5b50\u88fd\u5264\u306f\u3001\u73fe\u4ee3\u306e\u533b\u7642\u3092\u884c\u3046\u4e0a\u3067\u5fc5\u8981\u3068\u3055\u308c\u308b\u57fa\u672c\u7684\u306a\u85ac\u306e\u30ea\u30b9\u30c8\u3067\u3042\u308b\u3001WHO\u5fc5\u9808\u533b\u85ac\u54c1\u30e2\u30c7\u30eb\u30fb\u30ea\u30b9\u30c8\u306b\u6319\u3052\u3089\u308c\u3066\u3044\u308b[10]\u3002 \u305f\u3060\u3057\u3001\u8840\u6f3f\u4e2d\u306b\u304a\u3051\u308b\u7b2cVIII\u56e0\u5b50\u306e\u6fc3\u5ea6\u304c\u9ad8\u3051\u308c\u3070\u9ad8\u3044\u307b\u3069\u826f\u3044\u3068\u3044\u3046\u308f\u3051\u3067\u306f\u306a\u3044\u3002\u7b2cVIII\u56e0\u5b50\u306e\u6fc3\u5ea6\u304c\u9ad8\u3044\u30d2\u30c8\u306f\u3001\u6df1\u90e8\u9759\u8108\u8840\u6813\u75c7\u3068\u80ba\u8840\u6813\u585e\u6813\u75c7\u306e\u30ea\u30b9\u30af\u304c\u9ad8\u3044\u3053\u3068\u304c\u77e5\u3089\u308c\u3066\u3044\u308b[11]\u3002 \u30d2\u30c8\u3067\u306f\u3001F8\u907a\u4f1d\u5b50\u306fX\u67d3\u8272\u4f53\u306eq28\u306b\u4f4d\u7f6e\u3057\u3066\u3044\u308b\u3002\u7b2cVIII\u56e0\u5b50\u306f\u30011984\u5e74\u306b\u30b8\u30a7\u30cd\u30f3\u30c6\u30c3\u30af\u306e\u79d1\u5b66\u8005\u306b\u3088\u3063\u3066\u7279\u5fb4\u304c\u660e\u3089\u304b\u306b\u3055\u308c\u305f[12]\u3002\u7b2cVIII\u56e0\u5b50\u306e\u907a\u4f1d\u5b50\u306fX\u67d3\u8272\u4f53 (Xq28) \u306b\u4f4d\u7f6e\u3057\u3066\u3044\u308b\u3002\u7b2cVIII\u56e0\u5b50\u306e\u907a\u4f1d\u5b50\u306f\u3001\u30a4\u30f3\u30c8\u30ed\u30f3\u306e1\u3064\u306b\u5225\u306e\u907a\u4f1d\u5b50\u304c\u57cb\u3081\u8fbc\u307e\u308c\u3066\u3044\u308b\u3068\u3044\u3046\u3001\u8208\u5473\u6df1\u3044\u4e00\u6b21\u69cb\u9020\u3092\u6301\u3064[13]\u3002\u7b2cVIII\u56e0\u5b50\u306f\u3001A1-A2-B-A3-C1-C2\u3068\u3044\u30466\u3064\u306e\u30c9\u30e1\u30a4\u30f3\u304b\u3089\u69cb\u6210\u3055\u308c\u3001\u7b2cV\u56e0\u5b50\u3068\u76f8\u540c\u3067\u3042\u308b\u3002 A\u30c9\u30e1\u30a4\u30f3\u306f\u3001\u9285\u7d50\u5408\u30bf\u30f3\u30d1\u30af\u8cea\u306e\u30bb\u30eb\u30ed\u30d7\u30e9\u30b9\u30df\u30f3\u306eA\u30c9\u30e1\u30a4\u30f3\u3068\u76f8\u540c\u3067\u3042\u308b[14]\u3002C\u30c9\u30e1\u30a4\u30f3\u306f\u30ea\u30f3\u8102\u8cea\u7d50\u5408\u6027\u306e\u30c7\u30a3\u30b9\u30b3\u30a4\u30b8\u30f3\u30c9\u30e1\u30a4\u30f3\uff08\u82f1\u8a9e\u7248\uff09\u30d5\u30a1\u30df\u30ea\u30fc\u306b\u5c5e\u3057\u3001C2\u30c9\u30e1\u30a4\u30f3\u306f\u819c\u3078\u306e\u7d50\u5408\u3092\u62c5\u3046[15]\u3002\u7b2cVIII\u56e0\u5b50\u304b\u3089VIIIa\u56e0\u5b50\u3078\u306e\u6d3b\u6027\u5316\u306f\u3001B\u30c9\u30e1\u30a4\u30f3\u306e\u5207\u65ad\u3068\u653e\u51fa\u306b\u3088\u3063\u3066\u884c\u308f\u308c\u308b\u3002\u305d\u306e\u7d50\u679c\u3001\u30bf\u30f3\u30d1\u30af\u8cea\u306fA1-A2\u30c9\u30e1\u30a4\u30f3\u304b\u3089\u306a\u308b\u91cd\u9396\u3068A3-C1-C2\u30c9\u30e1\u30a4\u30f3\u304b\u3089\u306a\u308b\u8efd\u9396\u306b\u5206\u5272\u3055\u308c\u3001\u53cc\u65b9\u306f\u30ab\u30eb\u30b7\u30a6\u30e0\u4f9d\u5b58\u7684\u306b\u975e\u5171\u6709\u7d50\u5408\u6027\u306e\u8907\u5408\u4f53\u3092\u5f62\u6210\u3059\u308b\u3002\u3053\u306e\u8907\u5408\u4f53\u304c\u51dd\u56fa\u4fc3\u9032\u56e0\u5b50VIIIa\u3067\u3042\u308b[16]\u3002\u7b2cVIII\u56e0\u5b50\u306f\u7cd6\u30bf\u30f3\u30d1\u30af\u8cea\u3067\u3042\u308a\u3001\u88dc\u56e0\u5b50\u524d\u99c6\u4f53\u3067\u3042\u308b\u3002\u30d2\u30c8\u306b\u304a\u3051\u308b\u8840\u6d41\u3078\u306e\u653e\u51fa\u306e\u4e3b\u8981\u90e8\u4f4d\u306f\u6b63\u78ba\u306b\u306f\u5224\u3063\u3066\u3044\u306a\u3044\u3082\u306e\u306e\u3001\u8840\u7ba1\u3068\u7cf8\u7403\u4f53\u3068\u5c3f\u7d30\u7ba1\u306e\u5185\u76ae\u7d30\u80de\u3001\u809d\u81d3\u306e\u985e\u6d1e\u7d30\u80de\u3067\u5408\u6210\u3055\u308c\u8840\u6d41\u3078\u653e\u51fa\u3055\u308c\u308b[17]\u3002\u907a\u4f1d\u5b50\u7570\u5e38\u306b\u3088\u3063\u3066\u7b2cVIII\u56e0\u5b50\u304c\u4f5c\u308c\u306a\u3044\u305f\u3081\u306b\u8d77\u3053\u308b\u8840\u53cb\u75c5A\u306f\u3001\u809d\u79fb\u690d\u306b\u3088\u3063\u3066\u56de\u5fa9\u3059\u308b\u3053\u3068\u304c\u77e5\u3089\u308c\u3066\u3044\u308b[18]\u3002\u809d\u7d30\u80de\u306e\u79fb\u690d\u306f\u52b9\u679c\u304c\u306a\u3044\u304c\u3001\u809d\u81d3\u306e\u5185\u76ae\u7d30\u80de\u306e\u79fb\u690d\u306f\u52b9\u679c\u304c\u3042\u308b[18]\u3002\u3053\u306e\u3088\u3046\u306b\u809d\u81d3\u304c\u7b2cVIII\u56e0\u5b50\u306e\u7523\u751f\u306b\u5f37\u304f\u95a2\u4e0e\u3057\u3066\u3044\u308b\u3088\u3046\u306b\u898b\u3048\u308b\u3002\u3057\u304b\u3057\u306a\u304c\u3089\u3001\u809d\u81d3\u304c\u6a5f\u80fd\u4f4e\u4e0b\u3059\u308b\u3068\u3001\u4e00\u822c\u306b\u809d\u81d3\u3067\u5408\u6210\u3055\u308c\u308b\u30bf\u30f3\u30d1\u30af\u8cea\u3082\u91cf\u304c\u6e1b\u3063\u3066\u304f\u308b\u306e\u306b\u5bfe\u3057\u3066\u3001\u7b2cVIII\u56e0\u5b50\u306f\u809d\u81d3\u75c5\u306e\u5f71\u97ff\u3092\u53d7\u3051\u306a\u3044\u3002\u3080\u3057\u308d\u3001\u809d\u81d3\u304c\u6a5f\u80fd\u4f4e\u4e0b\u3057\u305f\u72b6\u6cc1\u4e0b\u3067\u306f\u3001\u7b2cVIII\u56e0\u5b50\u306e\u8840\u6f3f\u4e2d\u306e\u6fc3\u5ea6\u306f\u3001\u591a\u304f\u306e\u5834\u5408\u3067\u4e0a\u6607\u3057\u3066\u3044\u308b[19][20]\u3002\u7b2cVIII\u56e0\u5b50\u306f\u3001\u8840\u4e2d\u3067\u4e3b\u306bvon Willebrand\u56e0\u5b50\u3068\u5b89\u5b9a\u306a\u975e\u5171\u6709\u7d50\u5408\u6027\u306e\u8907\u5408\u4f53\u3092\u5f62\u6210\u3057\u3066\u5faa\u74b0\u3059\u308b[5]\u3002\u30c8\u30ed\u30f3\u30d3\u30f3 (\u7b2cIIa\u56e0\u5b50) 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Willebrand\u56e0\u5b50\u306b\u3088\u3063\u3066\u4fdd\u8b77\u3055\u308c\u3066\u3044\u306a\u3044\u306e\u3067\u3001\u4e3b\u306b\u6d3b\u6027\u5316\u3055\u308c\u305f\u30d7\u30ed\u30c6\u30a4\u30f3C\u3068\u7b2cIXa\u56e0\u5b50\u306b\u3088\u308b\u30bf\u30f3\u30d1\u30af\u8cea\u5206\u89e3\u306b\u3088\u3063\u3066\u4e0d\u6d3b\u6027\u5316\u3055\u308c\u3001\u8840\u6d41\u4e2d\u304b\u3089\u7d20\u65e9\u304f\u9664\u53bb\u3055\u308c\u308b\u3002\u3053\u308c\u306b\u3088\u3063\u3066\u8840\u6db2\u51dd\u56fa\u53cd\u5fdc\u304c\u7570\u5e38\u306b\u4ea2\u9032\u3059\u308b\u3053\u3068\u3092\u9632\u3044\u3067\u3044\u308b\u3002\u7b2cVIII\u56e0\u5b50\u306f\u9285\u7d50\u5408\u30bf\u30f3\u30d1\u30af\u8cea\u3067\u3042\u308b\u4e00\u65b9\u3001\u9285\u306e\u6b20\u4e4f\u306f\u7b2cVIII\u56e0\u5b50\u306e\u6d3b\u6027\u306e\u5897\u5927\u3092\u3082\u305f\u3089\u3059\u3053\u3068\u304c\u5831\u544a\u3055\u308c\u3066\u304a\u308a\u3001\u3053\u308c\u306f\u30d7\u30ed\u30c6\u30a4\u30f3C\u306e\u6d3b\u6027\u304c\u76f8\u5bfe\u7684\u306b\u4f4e\u4e0b\u3059\u308b\u305f\u3081\u3068\u63a8\u6e2c\u3055\u308c\u3066\u3044\u308b[21]\u3002Table of Contents\u533b\u7642\u306b\u304a\u3051\u308b\u5229\u7528[\u7de8\u96c6]\u6c5a\u67d3\u30b9\u30ad\u30e3\u30f3\u30c0\u30eb[\u7de8\u96c6]\u6ce8\u91c8[\u7de8\u96c6]\u51fa\u5178[\u7de8\u96c6]\u95a2\u9023\u6587\u732e[\u7de8\u96c6]\u5916\u90e8\u30ea\u30f3\u30af[\u7de8\u96c6]\u533b\u7642\u306b\u304a\u3051\u308b\u5229\u7528[\u7de8\u96c6]\u732e\u8840\u7531\u6765\u306e\u8840\u6f3f\u304b\u3089\u6fc3\u7e2e\u3059\u308b\u3053\u3068\u306b\u3088\u3063\u3066\u88fd\u9020\u3055\u308c\u305f\u7b2cVIII\u56e0\u5b50\u88fd\u5264\u304b\u3001\u3082\u3057\u304f\u306f\u907a\u4f1d\u5b50\u7d44\u63db\u3048\u7b2cVIII\u56e0\u5b50\u88fd\u5264\u304c\u3001\u8840\u53cb\u75c5A\u60a3\u8005\u306e\u8840\u6db2\u51dd\u56fa\u80fd\u3092\u4fdd\u3064\u305f\u3081\u3001\u5bfe\u75c7\u7642\u6cd5\u3068\u3057\u3066\u7e70\u308a\u8fd4\u3057\u9759\u8108\u5185\u6295\u4e0e\u3055\u308c\u308b\u3002\u3064\u307e\u308a\u3001\u7b2cVIII\u56e0\u5b50\u304c\u4e0d\u8db3\u3057\u3066\u3044\u308b\u305f\u3081\u3001\u305d\u306e\u88dc\u5145\u7642\u6cd5\u3092\u5b9a\u671f\u7684\u306b\u884c\u3063\u3066\u3044\u308b\u306e\u3067\u3042\u308b\u3002\u3057\u304b\u3057\u306a\u304c\u3089\u3001\u732e\u8840\u7531\u6765\u306e\u8840\u6f3f\u304b\u3089\u6fc3\u7e2e\u3059\u308b\u3053\u3068\u306b\u3088\u3063\u3066\u88fd\u9020\u3055\u308c\u305f\u7b2cVIII\u56e0\u5b50\u88fd\u5264\u306e\u5834\u5408\u306f\u3001\u30d2\u30c8\u7531\u6765\u306e\u8840\u6db2\u88fd\u5264\u3067\u3042\u308b\u305f\u3081\u3001HIV\u30a6\u30a4\u30eb\u30b9\u306a\u3069\u304c\u6f5c\u3093\u3067\u3044\u308b\u53ef\u80fd\u6027\u304c\u3042\u308a\u3001\u88dc\u5145\u7642\u6cd5\u3092\u53d7\u3051\u305f\u60a3\u8005\u306f\u3001\u8840\u6db2\u306b\u3088\u3063\u3066\u611f\u67d3\u3059\u308b\u69d8\u3005\u306a\u611f\u67d3\u75c7\u306b\u304b\u304b\u308b\u5371\u967a\u6027\u304c\u3042\u308b\u3002\u4f8b\u3048\u3070\u85ac\u5bb3\u30a8\u30a4\u30ba\u4e8b\u4ef6\u304c\u3053\u308c\u3067\u3042\u308b\u3002\u3053\u306e\u70b9\u306b\u304a\u3044\u3066\u3001\u30d2\u30c8\u306e\u8840\u6db2\u306b\u7531\u6765\u3057\u306a\u3044\u907a\u4f1d\u5b50\u7d44\u63db\u3048\u578b\u7b2cVII\u56e0\u5b50\u88fd\u5264\u306f\u5b89\u5168\u3068\u8a00\u3048\u308b\u3002\u305f\u3060\u3057\u3001\u3044\u305a\u308c\u306e\u7b2cVIII\u56e0\u5b50\u88fd\u5264\u3082\u8840\u53cb\u75c5A\u60a3\u8005\u306b\u3068\u3063\u3066\u306f\u7570\u7269\u3067\u3042\u308b\u305f\u3081\u3001\u6295\u4e0e\u3055\u308c\u305f\u7b2cVIII\u56e0\u5b50\u306b\u5bfe\u3059\u308b\u6297\u4f53\u304c\u5f62\u6210\u3055\u308c\u308b\u3068\u3044\u3046\u4e8b\u614b\u304c\u8d77\u3053\u308a\u5f97\u308b\u3002\u3053\u306e\u6297\u4f53\u5f62\u6210\u304c\u3001\u7b2cVIII\u56e0\u5b50\u88dc\u5145\u7642\u6cd5\u3092\u53d7\u3051\u3066\u3044\u308b\u60a3\u8005\u306b\u3068\u3063\u3066\u3001\u4e3b\u8981\u306a\u61f8\u5ff5\u4e8b\u9805\u3068\u306a\u3063\u3066\u3044\u308b\u3002\u6297\u4f53\u304c\u5f62\u6210\u3055\u308c\u308b\u3068\u3001\u7b2cVIII\u56e0\u5b50\u3092\u88dc\u5145\u3057\u3066\u3082\u6b21\u3005\u3068\u6297\u4f53\u306b\u3088\u3063\u3066\u4e0d\u6d3b\u5316\u3055\u308c\u3001\u52b9\u304b\u306a\u304f\u306a\u3063\u3066\u3086\u304f\u304b\u3089\u3067\u3042\u308b\u3002\u3053\u306e\u3088\u3046\u306a\u6297\u4f53\u306e\u767a\u751f\u306f\u3001\u7b2cVIII\u56e0\u5b50\u88fd\u5264\u81ea\u4f53\u3092\u542b\u3080\u3055\u307e\u3056\u307e\u306a\u56e0\u5b50\u304c\u539f\u56e0\u3068\u306a\u3063\u3066\u5f62\u6210\u3055\u308c\u308b[22]\u3002\u306a\u304a\u3001\u3053\u306e\u3088\u3046\u306a\u3053\u3068\u3082\u3042\u308a\u3001\u7279\u306b\u4f4e\u3044\u306a\u304c\u3089\u3082\u7b2cVIII\u56e0\u5b50\u7523\u751f\u80fd\u529b\u3092\u6301\u3063\u305f\u8840\u53cb\u75c5A\u306e\u8efd\u75c7\u60a3\u8005\u306e\u5834\u5408\u306f\u3001\u7b2cVIII\u56e0\u5b50\u306e\u653e\u51fa\u3092\u4fc3\u9032\u3059\u308b\u4f5c\u7528\u3092\u6301\u3063\u305f\u30c7\u30b9\u30e2\u30d7\u30ec\u30b7\u30f3\u3092\u6295\u4e0e\u3059\u308b\u3053\u3068\u3082\u3042\u308b\u3002\u6c5a\u67d3\u30b9\u30ad\u30e3\u30f3\u30c0\u30eb[\u7de8\u96c6]1980\u5e74\u4ee3\u306b\u30d0\u30af\u30b9\u30bf\u30fc\u3084\u30d0\u30a4\u30a8\u30eb\u3068\u3044\u3063\u305f\u4e00\u90e8\u306e\u88fd\u85ac\u4f01\u696d\u304c\u3001\u65b0\u305f\u306a\u52a0\u71b1\u88fd\u5264\u304c\u5229\u7528\u53ef\u80fd\u306b\u306a\u3063\u305f\u5f8c\u3082\u3001\u6c5a\u67d3\u3055\u308c\u305f\u7b2cVIII\u56e0\u5b50\u88fd\u5264\u306e\u8ca9\u58f2\u3092\u7d99\u7d9a\u3057\u305f\u3053\u3068\u306b\u5bfe\u3057\u3066\u8ad6\u4e89\u304c\u8d77\u3053\u3063\u305f[23]\u3002\u30a2\u30e1\u30ea\u30ab\u98df\u54c1\u533b\u85ac\u54c1\u5c40 (FDA) \u306e\u5727\u529b\u306e\u3082\u3068\u3001\u975e\u52a0\u71b1\u88fd\u5264\u306f\u30a2\u30e1\u30ea\u30ab\u306e\u5e02\u5834\u304b\u3089\u5f15\u304d\u63da\u3052\u3089\u308c\u305f\u3082\u306e\u306e\u3001\u30a2\u30b8\u30a2\u3001\u30e9\u30c6\u30f3\u30a2\u30e1\u30ea\u30ab\u3001\u30e8\u30fc\u30ed\u30c3\u30d1\u306e\u4e00\u90e8\u306e\u56fd\u3067\u306f\u8ca9\u58f2\u304c\u7d99\u7d9a\u3055\u308c\u305f\u3002\u305d\u306e\u88fd\u54c1\u306f\u3001\u30d0\u30a4\u30a8\u30eb\u3068FDA\u3068\u306e\u8b70\u8ad6\u3067\u61f8\u5ff5\u3055\u308c\u3066\u3044\u305f\u3001\u30d2\u30c8\u514d\u75ab\u4e0d\u5168\u30a6\u30a4\u30eb\u30b9\u306b\u3088\u3063\u3066\u6c5a\u67d3\u3055\u308c\u3066\u3044\u305f[23]\u30021990\u5e74\u4ee3\u521d\u982d\u306b\u88fd\u85ac\u4f01\u696d\u306f\u907a\u4f1d\u5b50\u7d44\u63db\u3048\u306b\u3088\u3063\u3066\u5408\u6210\u3055\u308c\u305f\u88fd\u54c1\u306e\u751f\u7523\u3092\u958b\u59cb\u3057\u3001\u73fe\u5728\u3067\u306f\u7b2cVIII\u56e0\u5b50\u88dc\u5145\u7642\u6cd5\u306b\u3088\u308b\u75c5\u6c17\u306e\u611f\u67d3\u306f\u3001\u307b\u307c\u5168\u3066\u306e\u5f62\u614b\u3067\u9632\u6b62\u3055\u308c\u3066\u3044\u308b\u3002\u6ce8\u91c8[\u7de8\u96c6]^ \u306a\u304a\u3001\u8840\u53cb\u75c5B\u306f\u7b2cIX\u56e0\u5b50\u3092\u30b3\u30fc\u30c9\u3059\u308b\u907a\u4f1d\u5b50\u306b\u554f\u984c\u304c\u3042\u308b\u305f\u3081\u306b\u767a\u751f\u3059\u308b\u3002\u305f\u3060\u3057\u3001\u7b2cIX\u56e0\u5b50\u306f\u6297\u8840\u53cb\u75c5\u56e0\u5b50B\u3068\u547c\u3070\u308c\u308b\u4ee5\u5916\u306b\u3082\u3001\u30af\u30ea\u30b9\u30de\u30b9\u56e0\u5b50\u306a\u3069\u3068\u3082\u547c\u3070\u308c\u308b\u3002\u51fa\u5178[\u7de8\u96c6]^ a b c GRCh38: Ensembl release 89: ENSG00000185010 – Ensembl, May 2017^ a b c GRCm38: Ensembl release 89: ENSMUSG00000031196 – Ensembl, May 2017^ Human PubMed Reference:^ Mouse PubMed Reference:^ a b c Reference, Genetics Home. \u201cF8 gene\u201d (\u82f1\u8a9e). Genetics Home Reference. 2019\u5e743\u670810\u65e5\u95b2\u89a7\u3002^ Toole JJ, Knopf JL, Wozney JM, Sultzman LA, Buecker JL, Pittman DD, Kaufman RJ, Brown E, Shoemaker C, Orr EC (1984). \u201cMolecular cloning of a cDNA encoding human antihaemophilic factor\u201d. Nature 312 (5992): 342-7. doi:10.1038\/312342a0. PMID\u00a06438528.\u00a0^ Truett MA, Blacher R, Burke RL, Caput D, Chu C, Dina D, Hartog K, Kuo CH, Masiarz FR, Merryweather JP (October 1985). \u201cCharacterization of the polypeptide composition of human factor VIII:C and the nucleotide sequence and expression of the human kidney cDNA\u201d. Dna 4 (5): 333-49. doi:10.1089\/dna.1985.4.333. PMID\u00a03935400.\u00a0^ \u201cF8 coagulation factor VIII [Homo sapiens (human) – Gene – NCBI]\u201d. www.ncbi.nlm.nih.gov. 2019\u5e743\u670810\u65e5\u95b2\u89a7\u3002^ Antonarakis SE (July 1995). \u201cMolecular genetics of coagulation factor VIII gene and hemophilia A\u201d. Thrombosis and Haemostasis 74 (1): 322-8. PMID\u00a08578479.\u00a0^ \u201c19th WHO Model List of Essential Medicines (April 2015)\u201d. WHO (2015\u5e744\u6708). 2015\u5e745\u670810\u65e5\u95b2\u89a7\u3002^ Jenkins PV, Rawley O, Smith OP, O’Donnell JS (June 2012). \u201cElevated factor VIII levels and risk of venous thrombosis\u201d. British Journal of Haematology 157 (6): 653-63. doi:10.1111\/j.1365-2141.2012.09134.x. PMID\u00a022530883.\u00a0^ Gitschier J, Wood WI, Goralka TM, Wion KL, Chen EY, Eaton DH, Vehar GA, Capon DJ, Lawn RM (November 1984). \u201cCharacterization of the human factor VIII gene\u201d. Nature 312 (5992): 326-30. PMID\u00a06438525.\u00a0^ Levinson B, Kenwrick S, Lakich D, Hammonds G, Gitschier J (May 1990). \u201cA transcribed gene in an intron of the human factor VIII gene\u201d. Genomics 7 (1): 1-11. doi:10.1016\/0888-7543(90)90512-S. PMID\u00a02110545.\u00a0^ Villoutreix BO, Dahlb\u00e4ck B (June 1998). \u201cStructural investigation of the A domains of human blood coagulation factor V by molecular modeling\u201d. Protein Science 7 (6): 1317-25. doi:10.1002\/pro.5560070607. PMC\u00a02144041. PMID\u00a09655335. https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC2144041\/.\u00a0^ Macedo-Ribeiro S, Bode W, Huber R, Quinn-Allen MA, Kim SW, Ortel TL, Bourenkov GP, Bartunik HD, Stubbs MT, Kane WH, Fuentes-Prior P (November 1999). \u201cCrystal structures of the membrane-binding C2 domain of human coagulation factor V\u201d. Nature 402 (6760): 434-439. doi:10.1038\/46594. PMID\u00a010586886.\u00a0^ Thorelli E, Kaufman RJ, Dahlb\u00e4ck B (June 1998). \u201cThe C-terminal region of the factor V B-domain is crucial for the anticoagulant activity of factor V\u201d. The Journal of Biological Chemistry 273 (26): 16140-16145. doi:10.1074\/jbc.273.26.16140. PMID\u00a09632668.\u00a0^ Kumar; Abbas; Fausto (2005). Robbins and Cotran Pathologic Basis of Disease. Pennsylvania: Elsevier. p.\u00a0655. ISBN\u00a01-889325-04-X\u00a0^ a b Kaushansky K, Lichtman M, Beutler E, Kipps T, Prchal J, Seligsohn U. (2010; edition 8) Williams Hematology. McGraw-Hill. 978-0-07-162151-9^ Hollestelle MJ, Geertzen HG, Straatsburg IH, van Gulik TM, van Mourik JA (February 2004). \u201cFactor VIII expression in liver disease\u201d. Thrombosis and Haemostasis 91 (2): 267-75. doi:10.1160\/th03-05-0310. PMID\u00a014961153.\u00a0^ R. Rubin; L. Leopold (1998). Hematologic Pathophysiology. Madison, Conn: Fence Creek Publishing. ISBN\u00a01-889325-04-X\u00a0^ Milne DB, Nielsen FH (March 1996). \u201cEffects of a diet low in copper on copper-status indicators in postmenopausal women\u201d. The American Journal of Clinical Nutrition 63 (3): 358-64. PMID\u00a08602593.\u00a0^ Lozier J (2004\u5e74). \u201cOverview of Factor VIII Inhibitors\u201d. CMEonHemophilia.com. 2008\u5e7412\u670816\u65e5\u6642\u70b9\u306e\u30aa\u30ea\u30b8\u30ca\u30eb\u3088\u308a\u30a2\u30fc\u30ab\u30a4\u30d6\u30022009\u5e741\u67087\u65e5\u95b2\u89a7\u3002^ a b Bogdanich W, Koli E (2003\u5e745\u670822\u65e5). \u201c2 Paths of Bayer Drug in 80’s: Riskier One Steered Overseas\u201d. The New York Times. 2009\u5e741\u67087\u65e5\u95b2\u89a7\u3002\u95a2\u9023\u6587\u732e[\u7de8\u96c6]Gitschier J (1991). \u201cThe molecular basis of hemophilia A\u201d. Annals of the New York Academy of Sciences 614 (1 Process in Va): 89-96. doi:10.1111\/j.1749-6632.1991.tb43694.x. PMID\u00a01902642.\u00a0White GC, Shoemaker CB (January 1989). \u201cFactor VIII gene and hemophilia A\u201d. Blood 73 (1): 1-12. PMID\u00a02491949.\u00a0Antonarakis SE, Kazazian HH, Tuddenham EG (1995). \u201cMolecular etiology of factor VIII deficiency in hemophilia A\u201d. Human Mutation 5 (1): 1-22. doi:10.1002\/humu.1380050102. PMID\u00a07728145.\u00a0Lenting PJ, van Mourik JA, Mertens K (December 1998). \u201cThe life cycle of coagulation factor VIII in view of its structure and function\u201d. Blood 92 (11): 3983-96. PMID\u00a09834200.\u00a0Saenko EL, Ananyeva N, Kouiavskaia D, Schwinn H, Josic D, Shima M, Hauser CA, Pipe S (August 2002). \u201cMolecular defects in coagulation Factor VIII and their impact on Factor VIII function\u201d. Vox Sanguinis 83 (2): 89-96. doi:10.1046\/j.1423-0410.2002.00183.x. PMID\u00a012201837.\u00a0Lollar P (August 2002). \u201cMolecular characterization of the immune response to factor VIII\u201d. Vox Sanguinis. 83 83 Suppl 1: 403-8. doi:10.1111\/j.1423-0410.2002.tb05342.x. PMID\u00a012617176.\u00a0Fay PJ (March 2004). \u201cActivation of factor VIII and mechanisms of cofactor action\u201d. Blood Reviews 18 (1): 1-15. doi:10.1016\/S0268-960X(03)00025-0. PMID\u00a014684146.\u00a0Lavigne-Lissalde G, Schved JF, Granier C, Villard S (October 2005). \u201cAnti-factor VIII antibodies: a 2005 update\u201d. Thrombosis and Haemostasis 94 (4): 760-9. doi:10.1160\/TH05-02-0118. PMID\u00a016270627.\u00a0Fang H, Wang L, Wang H (2007). \u201cThe protein structure and effect of factor VIII\u201d. Thrombosis Research 119 (1): 1-13. doi:10.1016\/j.thromres.2005.12.015. PMID\u00a016487577.\u00a0\u5916\u90e8\u30ea\u30f3\u30af[\u7de8\u96c6]"},{"@context":"http:\/\/schema.org\/","@type":"BreadcrumbList","itemListElement":[{"@type":"ListItem","position":1,"item":{"@id":"https:\/\/wiki.edu.vn\/jp\/wiki9\/#breadcrumbitem","name":"Enzyklop\u00e4die"}},{"@type":"ListItem","position":2,"item":{"@id":"https:\/\/wiki.edu.vn\/jp\/wiki9\/archives\/108320#breadcrumbitem","name":"\u7b2cVIII\u56e0\u5b50 – Wikipedia"}}]}]