[{"@context":"http:\/\/schema.org\/","@type":"BlogPosting","@id":"https:\/\/wiki.edu.vn\/jp\/wiki7\/2018\/09\/17\/%e3%83%9d%e3%83%aa%e3%82%b0%e3%83%ab%e3%82%bf%e3%83%9f%e3%83%b3%e7%b5%90%e5%90%88%e3%82%bf%e3%83%b3%e3%83%91%e3%82%af%e8%b3%aa-1-wikipedia\/#BlogPosting","mainEntityOfPage":"https:\/\/wiki.edu.vn\/jp\/wiki7\/2018\/09\/17\/%e3%83%9d%e3%83%aa%e3%82%b0%e3%83%ab%e3%82%bf%e3%83%9f%e3%83%b3%e7%b5%90%e5%90%88%e3%82%bf%e3%83%b3%e3%83%91%e3%82%af%e8%b3%aa-1-wikipedia\/","headline":"\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u7d50\u5408\u30bf\u30f3\u30d1\u30af\u8cea-1 – Wikipedia","name":"\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u7d50\u5408\u30bf\u30f3\u30d1\u30af\u8cea-1 – Wikipedia","description":"\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u7d50\u5408\u30bf\u30f3\u30d1\u30af\u8cea-1\uff08\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u3051\u3064\u3054\u3046\u30bf\u30f3\u30d1\u30af\u3057\u3064\u3001\u82f1: Polyglutamine-binding protein 1\u3001\u7565\u79f0 PQBP1\u3042\u308b\u3044\u306fPQBP-1\uff09\u306f\u3001\u30d2\u30c8\u3067\u306fPQBP1\u907a\u4f1d\u5b50\u306b\u3088\u3063\u3066\u30b3\u30fc\u30c9\u3055\u308c\u308b\u30bf\u30f3\u30d1\u30af\u8cea\u3067\u3042\u308b[5][6][7]\u3002\u306a\u304a\u3001\u4eba\u5de5\u7684\u306a\u5408\u6210\u30da\u30d7\u30c1\u30c9\u3067\u3042\u308bQBP1(polyglutamine binding peptide 1)\u3068\u306f\u5168\u304f\u95a2\u4fc2\u306f\u306a\u3044\u3002 PQBP1\u306f\u3001yeast two-hybrid\u6cd5\u306b\u3088\u3063\u3066\u8133\u7d44\u7e54\u7531\u6765\u306ecDNA library\u3092\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u7e70\u308a\u8fd4\u3057\u914d\u5217\u3092bait\u306b\u7528\u3044\u3066\u30b9\u30af\u30ea\u30fc\u30cb\u30f3\u30b0\u3059\u308b\u3053\u3068\u306b\u3088\u308a\u3001\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u914d\u5217\u306b\u7d50\u5408\u3059\u308b\u30bf\u30f3\u30d1\u30af\u8cea\u3068\u3057\u3066\u767a\u898b\u3055\u308c\u305f[8][9]\u3002PQBP1\u306emRNA\u304a\u3088\u3073\u30bf\u30f3\u30d1\u30af\u8cea\u306f\u5e83\u7bc4\u306a\u7d30\u80de\u30fb\u7d44\u7e54\u3067\u767a\u73fe\u3057\u3066\u304a\u308a\u3001\u7d30\u80de\u306b\u304a\u3044\u3066\u306f\u6838\u512a\u4f4d\u306b\u767a\u73fe\u3059\u308b\u304c\u7d30\u80de\u8cea\u306b\u3082\u5b58\u5728\u3057\u3066\u3044\u308b[10]\u3002\u7d44\u7e54\u306b\u304a\u3044\u3066\u306f\u3001\u5916\u80da\u8449[11]\u3001\u4e2d\u80da\u8449\u306a\u3069\u3067\u767a\u73fe\u3059\u308b[12]\u3002\u30de\u30a6\u30b9\u306a\u3069\u54fa\u4e73\u985e\u306e\u8133\u767a\u751f\u904e\u7a0b\u306b\u304a\u3044\u3066\u306f\u3001ventricular zone\u304a\u3088\u3073subventricular zone\uff08\u8133\u5ba4\u4e0b\u5e2f\uff09\u306b\u9ad8\u3044\u767a\u73fe\u3092\u8a8d\u3081\u308b[13][14]\u3002PQBP1\u306f\u9032\u5316\u306e\u904e\u7a0b\u3067\u9ad8\u5ea6\u306b\u4fdd\u5b58\u3055\u308c\u3066\u304a\u308a\u3001PQBP1\u306f\u3001\u970a\u9577\u985e\u3001\u54fa\u4e73\u985e\u304b\u3089\u3001\u30b7\u30ed\u30a4\u30cc\u30ca\u30ba\u30ca\u306a\u3069\u306e\u690d\u7269\u3001\u7dda\u866b\u306a\u3069\u306e\u4e0b\u7b49\u52d5\u7269\u307e\u3067\u3001\u7a2e\u3092\u8d85\u3048\u3066\u5b58\u5728\u3057\u3066\u3044\u308b[15]\u3002 Table of Contents \u5206\u5b50\u6a5f\u80fd[\u7de8\u96c6]\u751f\u7269\u5b66\u7684\u6a5f\u80fd[\u7de8\u96c6]\u81e8\u5e8a\u7684\u610f\u7fa9[\u7de8\u96c6]\u30e2\u30c7\u30eb\u52d5\u7269[\u7de8\u96c6]\u76f8\u4e92\u4f5c\u7528[\u7de8\u96c6]\u63a8\u85a6\u6587\u732e[\u7de8\u96c6]\u5916\u90e8\u30ea\u30f3\u30af[\u7de8\u96c6] \u5206\u5b50\u6a5f\u80fd[\u7de8\u96c6] PQBP1\u306f\u3001WW\u30c9\u30e1\u30a4\u30f3\u3068C\u672b\u7aef\u30c9\u30e1\u30a4\u30f3\u3092\u542b\u3093\u3067\u304a\u308a\u3001\u305d\u306e\u4e2d\u9593\u306b\u306f\u30ea\u30d4\u30fc\u30c8\u914d\u5217\u3092\u6709\u3059\u308b[16][\u63d0\u4f9b\uff1aOMIM][17]\u3002C\u672b\u7aef\u30c9\u30e1\u30a4\u30f3\u306f\u5929\u7136\u5909\u6027\u30bf\u30f3\u30d1\u30af\u8cea\u306e\u6027\u8cea\u3092\u6301\u3063\u3066\u304a\u308a[18][19][20]\u3001\u56fa\u5b9a\u3055\u308c\u305f\u69cb\u9020\u3092\u53d6\u3089\u306a\u3044\u304c\u3001\u305d\u306e\u4e2d\u306e\u7279\u5b9a\u306a\u914d\u5217\u3092\u4f7f\u3063\u3066\u3001U5\u30b9\u30d7\u30e9\u30a4\u30b7\u30f3\u30b0\u8907\u5408\u4f53\u306e\u69cb\u6210\u8981\u7d20\u3067\u3042\u308bU5-15kD\u3068\u7d50\u5408\u3059\u308b[21][22][23]\u3002\u307e\u305f\u3001U2\u30b9\u30d7\u30e9\u30a4\u30b7\u30f3\u30b0\u8907\u5408\u4f53\u306e\u69cb\u6210\u8981\u7d20\u3068\u306e\u95a2\u9023\u3092\u793a\u3059\u5831\u544a\u3082\u3042\u308b[24]\u3002C\u672b\u7aef\u30c9\u30e1\u30a4\u30f3\u306f\u5929\u7136\u5909\u6027\u30bf\u30f3\u30d1\u30af\u8cea\u3068\u3057\u3066\u50cd\u304f\u305f\u3081\u3001\u7269\u7406\u5316\u5b66\u7684\u306a\u6027\u8cea\u3068\u3057\u3066\u6db2\u6db2\u76f8\u5206\u96e2\u3092\u8d77\u3053\u3057\u3001\u7d30\u80de\u6838\u5185\u90e8\u3067\u6838\u30de\u30c8\u30ea\u30c3\u30af\u30b9\u306bnuclear body\u3092\u5f62\u6210\u3059\u308b\u304c[25][26]\u3001\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u75c5\u306e\uff11\u3064\u3067\u3042\u308b\u810a\u9ac4\u5c0f\u8133\u5931\u8abf\u75c7\uff11\u578b\u306e\u539f\u56e0\u30bf\u30f3\u30d1\u30af\u8ceaAtaxin-1\u3068\u76f8\u4e92\u4f5c\u7528\u3057\u3066\u3001nuclear","datePublished":"2018-09-17","dateModified":"2018-09-17","author":{"@type":"Person","@id":"https:\/\/wiki.edu.vn\/jp\/wiki7\/author\/lordneo\/#Person","name":"lordneo","url":"https:\/\/wiki.edu.vn\/jp\/wiki7\/author\/lordneo\/","image":{"@type":"ImageObject","@id":"https:\/\/secure.gravatar.com\/avatar\/c9645c498c9701c88b89b8537773dd7c?s=96&d=mm&r=g","url":"https:\/\/secure.gravatar.com\/avatar\/c9645c498c9701c88b89b8537773dd7c?s=96&d=mm&r=g","height":96,"width":96}},"publisher":{"@type":"Organization","name":"Enzyklop\u00e4die","logo":{"@type":"ImageObject","@id":"https:\/\/wiki.edu.vn\/wiki4\/wp-content\/uploads\/2023\/08\/download.jpg","url":"https:\/\/wiki.edu.vn\/wiki4\/wp-content\/uploads\/2023\/08\/download.jpg","width":600,"height":60}},"image":{"@type":"ImageObject","@id":"https:\/\/ja.wikipedia.org\/wiki\/Special:CentralAutoLogin\/start?type=1x1","url":"https:\/\/ja.wikipedia.org\/wiki\/Special:CentralAutoLogin\/start?type=1x1","height":"1","width":"1"},"url":"https:\/\/wiki.edu.vn\/jp\/wiki7\/2018\/09\/17\/%e3%83%9d%e3%83%aa%e3%82%b0%e3%83%ab%e3%82%bf%e3%83%9f%e3%83%b3%e7%b5%90%e5%90%88%e3%82%bf%e3%83%b3%e3%83%91%e3%82%af%e8%b3%aa-1-wikipedia\/","about":["Wiki"],"wordCount":20683,"articleBody":"\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u7d50\u5408\u30bf\u30f3\u30d1\u30af\u8cea-1\uff08\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u3051\u3064\u3054\u3046\u30bf\u30f3\u30d1\u30af\u3057\u3064\u3001\u82f1: Polyglutamine-binding protein 1\u3001\u7565\u79f0 PQBP1\u3042\u308b\u3044\u306fPQBP-1\uff09\u306f\u3001\u30d2\u30c8\u3067\u306fPQBP1\u907a\u4f1d\u5b50\u306b\u3088\u3063\u3066\u30b3\u30fc\u30c9\u3055\u308c\u308b\u30bf\u30f3\u30d1\u30af\u8cea\u3067\u3042\u308b[5][6][7]\u3002\u306a\u304a\u3001\u4eba\u5de5\u7684\u306a\u5408\u6210\u30da\u30d7\u30c1\u30c9\u3067\u3042\u308bQBP1(polyglutamine binding peptide 1)\u3068\u306f\u5168\u304f\u95a2\u4fc2\u306f\u306a\u3044\u3002  PQBP1\u306f\u3001yeast two-hybrid\u6cd5\u306b\u3088\u3063\u3066\u8133\u7d44\u7e54\u7531\u6765\u306ecDNA library\u3092\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u7e70\u308a\u8fd4\u3057\u914d\u5217\u3092bait\u306b\u7528\u3044\u3066\u30b9\u30af\u30ea\u30fc\u30cb\u30f3\u30b0\u3059\u308b\u3053\u3068\u306b\u3088\u308a\u3001\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u914d\u5217\u306b\u7d50\u5408\u3059\u308b\u30bf\u30f3\u30d1\u30af\u8cea\u3068\u3057\u3066\u767a\u898b\u3055\u308c\u305f[8][9]\u3002PQBP1\u306emRNA\u304a\u3088\u3073\u30bf\u30f3\u30d1\u30af\u8cea\u306f\u5e83\u7bc4\u306a\u7d30\u80de\u30fb\u7d44\u7e54\u3067\u767a\u73fe\u3057\u3066\u304a\u308a\u3001\u7d30\u80de\u306b\u304a\u3044\u3066\u306f\u6838\u512a\u4f4d\u306b\u767a\u73fe\u3059\u308b\u304c\u7d30\u80de\u8cea\u306b\u3082\u5b58\u5728\u3057\u3066\u3044\u308b[10]\u3002\u7d44\u7e54\u306b\u304a\u3044\u3066\u306f\u3001\u5916\u80da\u8449[11]\u3001\u4e2d\u80da\u8449\u306a\u3069\u3067\u767a\u73fe\u3059\u308b[12]\u3002\u30de\u30a6\u30b9\u306a\u3069\u54fa\u4e73\u985e\u306e\u8133\u767a\u751f\u904e\u7a0b\u306b\u304a\u3044\u3066\u306f\u3001ventricular zone\u304a\u3088\u3073subventricular zone\uff08\u8133\u5ba4\u4e0b\u5e2f\uff09\u306b\u9ad8\u3044\u767a\u73fe\u3092\u8a8d\u3081\u308b[13][14]\u3002PQBP1\u306f\u9032\u5316\u306e\u904e\u7a0b\u3067\u9ad8\u5ea6\u306b\u4fdd\u5b58\u3055\u308c\u3066\u304a\u308a\u3001PQBP1\u306f\u3001\u970a\u9577\u985e\u3001\u54fa\u4e73\u985e\u304b\u3089\u3001\u30b7\u30ed\u30a4\u30cc\u30ca\u30ba\u30ca\u306a\u3069\u306e\u690d\u7269\u3001\u7dda\u866b\u306a\u3069\u306e\u4e0b\u7b49\u52d5\u7269\u307e\u3067\u3001\u7a2e\u3092\u8d85\u3048\u3066\u5b58\u5728\u3057\u3066\u3044\u308b[15]\u3002Table of Contents\u5206\u5b50\u6a5f\u80fd[\u7de8\u96c6]\u751f\u7269\u5b66\u7684\u6a5f\u80fd[\u7de8\u96c6]\u81e8\u5e8a\u7684\u610f\u7fa9[\u7de8\u96c6]\u30e2\u30c7\u30eb\u52d5\u7269[\u7de8\u96c6]\u76f8\u4e92\u4f5c\u7528[\u7de8\u96c6]\u63a8\u85a6\u6587\u732e[\u7de8\u96c6]\u5916\u90e8\u30ea\u30f3\u30af[\u7de8\u96c6]\u5206\u5b50\u6a5f\u80fd[\u7de8\u96c6]PQBP1\u306f\u3001WW\u30c9\u30e1\u30a4\u30f3\u3068C\u672b\u7aef\u30c9\u30e1\u30a4\u30f3\u3092\u542b\u3093\u3067\u304a\u308a\u3001\u305d\u306e\u4e2d\u9593\u306b\u306f\u30ea\u30d4\u30fc\u30c8\u914d\u5217\u3092\u6709\u3059\u308b[16][\u63d0\u4f9b\uff1aOMIM][17]\u3002C\u672b\u7aef\u30c9\u30e1\u30a4\u30f3\u306f\u5929\u7136\u5909\u6027\u30bf\u30f3\u30d1\u30af\u8cea\u306e\u6027\u8cea\u3092\u6301\u3063\u3066\u304a\u308a[18][19][20]\u3001\u56fa\u5b9a\u3055\u308c\u305f\u69cb\u9020\u3092\u53d6\u3089\u306a\u3044\u304c\u3001\u305d\u306e\u4e2d\u306e\u7279\u5b9a\u306a\u914d\u5217\u3092\u4f7f\u3063\u3066\u3001U5\u30b9\u30d7\u30e9\u30a4\u30b7\u30f3\u30b0\u8907\u5408\u4f53\u306e\u69cb\u6210\u8981\u7d20\u3067\u3042\u308bU5-15kD\u3068\u7d50\u5408\u3059\u308b[21][22][23]\u3002\u307e\u305f\u3001U2\u30b9\u30d7\u30e9\u30a4\u30b7\u30f3\u30b0\u8907\u5408\u4f53\u306e\u69cb\u6210\u8981\u7d20\u3068\u306e\u95a2\u9023\u3092\u793a\u3059\u5831\u544a\u3082\u3042\u308b[24]\u3002C\u672b\u7aef\u30c9\u30e1\u30a4\u30f3\u306f\u5929\u7136\u5909\u6027\u30bf\u30f3\u30d1\u30af\u8cea\u3068\u3057\u3066\u50cd\u304f\u305f\u3081\u3001\u7269\u7406\u5316\u5b66\u7684\u306a\u6027\u8cea\u3068\u3057\u3066\u6db2\u6db2\u76f8\u5206\u96e2\u3092\u8d77\u3053\u3057\u3001\u7d30\u80de\u6838\u5185\u90e8\u3067\u6838\u30de\u30c8\u30ea\u30c3\u30af\u30b9\u306bnuclear body\u3092\u5f62\u6210\u3059\u308b\u304c[25][26]\u3001\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u75c5\u306e\uff11\u3064\u3067\u3042\u308b\u810a\u9ac4\u5c0f\u8133\u5931\u8abf\u75c7\uff11\u578b\u306e\u539f\u56e0\u30bf\u30f3\u30d1\u30af\u8ceaAtaxin-1\u3068\u76f8\u4e92\u4f5c\u7528\u3057\u3066\u3001nuclear body\u306e\u30b5\u30a4\u30ba\u3084\u5f62\u72b6\u304c\u5909\u5316\u3059\u308b\u3053\u3068\u304c\u77e5\u3089\u308c\u3066\u3044\u308b[27]\u3002WW\u30c9\u30e1\u30a4\u30f3\u306f\u3001RNA\u30dd\u30ea\u30e1\u30e9\u30fc\u30bcII\u306eC\u672b\u7aef\u9818\u57df\u306e\u7e70\u308a\u8fd4\u3057\u914d\u5217\u306b\u30ea\u30f3\u9178\u5316\u4f9d\u5b58\u7684\u306b\u7d50\u5408\u3059\u308b[27]\u3002\u3053\u308c\u3089\u306e\u3053\u3068\u304b\u3089\u3001PQBP1\u306fpre-mRNA\u8ee2\u5199\u76f4\u5f8c\u306e\u30b9\u30d7\u30e9\u30a4\u30b7\u30f3\u30b0\u3068\u306e\u30ab\u30c3\u30d7\u30ea\u30f3\u30b0\u6642\u306b\u50cd\u304f\u3068\u8003\u3048\u3089\u308c\u3001pre-mRNA\u30b9\u30d7\u30e9\u30a4\u30b7\u30f3\u30b0\u3068\u8ee2\u5199\u306b\u304a\u3044\u3066\u6a5f\u80fd\u3057\u3066\u3044\u308b\u3053\u3068\u304c\u308f\u304b\u3063\u3066\u3044\u308b\u3002  PQBP1\u306e\u7d30\u80de\u8cea\u5c40\u5728\u306b\u95a2\u9023\u3057\u305f\u5206\u5b50\u6a5f\u80fd\u3082\u5831\u544a\u3055\u308c\u3066\u3044\u308b\u3002PQBP1\u306f\u30b9\u30c8\u30ec\u30b9\u9846\u7c92\u306b\u5b58\u5728\u3057\u3001\u7d30\u80de\u8cea\u306b\u304a\u3051\u308bmRNA\u306e\u8caf\u8535\u5206\u89e3\u306b\u95a2\u4e0e\u3057\u3001\u8106\u5f31X\u75c7\u5019\u7fa4\u306e\u539f\u56e0\u30bf\u30f3\u30d1\u30af\u8cea\u3067\u3042\u308bFMRP\u3068\u3082\u5171\u540c\u7684\u306b\u6a5f\u80fd\u3059\u308b\u3053\u3068\u304c\u793a\u5506\u3055\u308c\u3066\u3044\u308b[28]\u3002\u307e\u305f\u3001PQBP1\u306f\u3001\u975e\u30ea\u30f3\u9178\u5316\u578b\u306e\u7ffb\u8a33\u4f38\u5f35\u56e0\u5b50Eukaryotic elongation factor 2 (eEF2) \u3068\u7d50\u5408\u3057\u3066\u3001eEF2\u306eThr56\u30ea\u30f3\u9178\u5316\u306b\u3088\u308b\u7ffb\u8a33\u4f38\u5f35\u6a5f\u80fd\u306e\u4f4e\u4e0b\u3092\u6291\u5236\u3059\u308b[29]\u3002\u3000\u3000\u751f\u7269\u5b66\u7684\u6a5f\u80fd[\u7de8\u96c6]\u4e0a\u8ff0\u306e\u3088\u3046\u306b\u3001PQBP1\u306ftranscriptional\u3042\u308b\u3044\u306fpost-transcriptional\u306b\u7a2e\u3005\u306emRNA\u304a\u3088\u3073\u30bf\u30f3\u30d1\u30af\u8cea\u306e\u767a\u73fe\u5236\u5fa1\u3092\u884c\u3063\u3066\u3044\u308b\u3002\u3057\u305f\u304c\u3063\u3066\u3001PQBP1\u306f\u3001\u767a\u73fe\u5236\u5fa1\u306e\u5bfe\u8c61\u3068\u306a\u308b\u907a\u4f1d\u5b50\u7fa4\u30fb\u30bf\u30f3\u30d1\u30af\u8cea\u7fa4\u306b\u57fa\u3065\u304f\u751f\u7269\u5b66\u7684\u6a5f\u80fd\u3092\u62c5\u3063\u3066\u3044\u308b\u3002PQBP1\u306f\u3001\u7d30\u80de\u5468\u671f\u30bf\u30f3\u30d1\u30af\u8cea\u3001\u7279\u306banaphase promoting complex (APC)\u30bf\u30f3\u30d1\u30af\u8cea\u306e\u767a\u73fe\u91cf\u5236\u5fa1\u3092\u4ecb\u3057\u3066\u3001\u795e\u7d4c\u5e79\u7d30\u80de\u306e\u7d30\u80de\u5468\u671f\u306b\u95a2\u308f\u3063\u3066\u3044\u308b[30]\u3002\u307e\u305f\u3001\u30b7\u30ca\u30d7\u30b9\u95a2\u9023\u30bf\u30f3\u30d1\u30af\u8cea\u3001\u795e\u7d4c\u7a81\u8d77\u95a2\u9023\u30bf\u30f3\u30d1\u30af\u8cea\u306e\u767a\u73fe\u91cf\u5236\u5fa1\u3092\u4ecb\u3057\u3066\u3001\u30b7\u30ca\u30d7\u30b9\u5f62\u6210\u304a\u3088\u3073\u795e\u7d4c\u7a81\u8d77\u4f38\u5f35\u306b\u95a2\u308f\u3063\u3066\u3044\u308b[31][32]\u3002\u307e\u305f\u3001\u7ffb\u8a33\u5236\u5fa1\u5236\u5fa1\u306e\u6a19\u7684\u6a5f\u80fd\u3068\u3057\u3066\u3001mGluR\u4f9d\u5b58\u7684\u306aLTD\u304c\u793a\u5506\u3055\u308c\u3066\u3044\u308b[33]\u3002\u81ea\u7136\u514d\u75ab\u3092\u62c5\u3046\u30de\u30af\u30ed\u30d5\u30a1\u30fc\u30b8\u3084\u30df\u30af\u30ed\u30b0\u30ea\u30a2\u3067\u306f\u3001PQBP1\u306f\u7d30\u80de\u8cea\u306b\u591a\u304f\u5b58\u5728\u3057\u3066\u3044\u308b\u3002PQBP1\u306f\u30a8\u30a4\u30ba\u30a6\u30a3\u30eb\u30b9(HIV1)\u306ecDNA\u306b\u5bfe\u3059\u308b\u7d30\u80de\u5185\u53d7\u5bb9\u4f53\u3068\u3057\u3066\u50cd\u304d[34]\u3001cGAS-STING\u7cfb\u306e\u7d30\u80de\u5185\u30b7\u30b0\u30ca\u30eb[35]\u3092\u901a\u3058\u3066IFN1\u306a\u3069\u3092\u8a98\u5c0e\u3057\u3066\u81ea\u7136\u514d\u75ab\u3092\u6d3b\u6027\u5316\u3059\u308b[36]\u3002\u8133\u306e\u30df\u30af\u30ed\u30b0\u30ea\u30a2\u3067\u306f\u3001\u540c\u69d8\u306b\u30bf\u30a6\u30bf\u30f3\u30d1\u30af\u8cea\u306b\u5bfe\u3059\u308b\u7d30\u80de\u5185\u53d7\u5bb9\u4f53\u3068\u3057\u3066\u50cd\u304d[37]\u3001cGAS-STING\u7cfb\u306e\u7d30\u80de\u5185\u30b7\u30b0\u30ca\u30eb[35]\u3092\u901a\u3058\u3066IFN1\u306a\u3069\u3092\u8a98\u5c0e\u3057\u3066\u81ea\u7136\u514d\u75ab\u3092\u6d3b\u6027\u5316\u3059\u308b[38]\u3002\u81e8\u5e8a\u7684\u610f\u7fa9[\u7de8\u96c6]\u3053\u306e\u30bf\u30f3\u30d1\u30af\u8cea\u3092\u30b3\u30fc\u30c9\u3059\u308bPQBP1\u907a\u4f1d\u5b50\u306e\u5909\u7570\u306f\u3001X\u9023\u9396\u52a3\u6027\u907a\u4f1d\u5f62\u5f0f\u306e\u30ec\u30f3\u30da\u30cb\u30f3\u30b0\u75c7\u5019\u7fa4\uff08\u82f1\u8a9e\u7248\uff09[39]\u3001Golabi-Ito-Hall \u75c7\u5019\u7fa4[40]\u3001Hamel \u75c7\u5019\u7fa4\u3001Proteus\u75c7\u5019\u7fa4\u3001Sutherland-Haan\u75c7\u5019\u7fa4\u306a\u3069\u306e\u8907\u6570\u75c7\u5019\u7fa4\u3092\u542b\u3080\u3001\u75c7\u5019\u6027\u77e5\u7684\u969c\u5bb3\uff08syndromic intellectual disability\uff09\u3042\u308b\u3044\u306f\u975e\u75c7\u5019\u6027\u77e5\u7684\u969c\u5bb3(non-syndromic intellectual disability)\u3092\u5f15\u304d\u8d77\u3053\u3059\u3053\u3068\u304c\u77e5\u3089\u308c\u3066\u3044\u308b[41][42]\u3002\u75c7\u5019\u6027\u77e5\u7684\u969c\u5bb3\u306e\u60a3\u8005\u306f\u3001\u5c0f\u982d\u75c7\u3001\u4f4e\u8eab\u9577\u3001\u77e5\u7684\u767a\u9054\u969c\u5bb3\u306a\u3069\u306e\u5171\u901a\u306e\u75c7\u72b6\u304c\u898b\u3089\u308c\u308b\u304c\u3001\u8eab\u4f53\u7684\u7279\u5fb4\u3092\u4f34\u308f\u306a\u3044\u975e\u75c7\u5019\u6027\u77e5\u7684\u969c\u5bb3\u306e\u539f\u56e0\u3068\u3082\u306a\u308a\u3046\u308b\u3002\u5c0f\u982d\u75c7\u3001\u9aa8\u683c\u306e\u4f4e\u5f62\u6210\u3001\u9854\u9762\u306a\u3069\u306e\u5c0f\u5947\u5f62\u4ee5\u5916\u306b\u3001\u8178\u306a\u3069\u306e\u5168\u8eab\u81d3\u5668\u306e\u7570\u5e38\u306e\u5831\u544a\u306f\u306a\u3044\u3002\u3053\u308c\u307e\u3067\u306b\u540c\u5b9a\u3055\u308c\u3066\u3044\u308b\u5909\u7570\u306f11\u7a2e\u985e\u3067\u3001\u6700\u3082\u983b\u5ea6\u306e\u9ad8\u3044\u3082\u306e\u306f\u30d5\u30ec\u30fc\u30e0\u30b7\u30d5\u30c8\u5909\u7570\u3067\u3042\u308b\u3002  \u307e\u305f\u3001\u30a2\u30eb\u30c4\u30cf\u30a4\u30de\u30fc\u75c5\u3001\u30bf\u30a6\u30aa\u30d1\u30c1\u30fc\u306a\u3069\u306e\u5909\u6027\u75be\u60a3[43][44]\u3001\u3042\u308b\u3044\u306fHIV\u306b\u5bfe\u3059\u308b\u514d\u75ab\u53cd\u5fdc[45][46]\u306a\u3069\u3001\u5f8c\u5929\u7684\u306a\u75c5\u614b\u6a5f\u80fd\u3078\u306e\u95a2\u4e0e\u304c\u3001\u30e2\u30c7\u30eb\u52d5\u7269\u304a\u3088\u3073\u30d2\u30c8\u60a3\u8005\u6b7b\u5f8c\u8133\u306e\u89e3\u6790\u7d50\u679c\u306a\u3069\u304b\u3089\u6307\u6458\u3055\u308c\u3066\u3044\u308b\u3002\u30e2\u30c7\u30eb\u52d5\u7269[\u7de8\u96c6]\u3053\u308c\u307e\u3067\u306b\u30de\u30a6\u30b9\u30e2\u30c7\u30eb\u3001\u30b7\u30e7\u30a6\u30b8\u30e7\u30a6\u30d0\u30a8\u30e2\u30c7\u30eb\u3001\u7dda\u866b\u30e2\u30c7\u30eb\u304c\u5831\u544a\u3055\u308c\u3066\u3044\u308b\u3002\u30de\u30a6\u30b9\u3067\u306f\u3001\u30c8\u30e9\u30f3\u30b9\u30b8\u30a7\u30cb\u30c3\u30af\u30de\u30a6\u30b9\u3001\u30ce\u30c3\u30af\u30c0\u30a6\u30f3\u30de\u30a6\u30b9\u3001\u30b3\u30f3\u30c7\u30a3\u30b7\u30e7\u30ca\u30eb\u30ce\u30c3\u30af\u30a2\u30a6\u30c8\u30de\u30a6\u30b9\u304c\u4f5c\u6210\u30fb\u5831\u544a\u3055\u308c\u3066\u3044\u308b\u3002\u30c8\u30e9\u30f3\u30b9\u30b8\u30a7\u30cb\u30c3\u30af\u30de\u30a6\u30b9\u3067\u306f\u3001F0\u30de\u30a6\u30b9\uff12\u5339\u306e\u3046\u3061\uff11\u5339\u306b\u304a\u3044\u3066\u3001\u9045\u767a\u6027\u306e\u904b\u52d5\u30cb\u30e5\u30fc\u30ed\u30f3\u969c\u5bb3\u304c\u8a8d\u3081\u3089\u308c\u305f[47]\u3002\u4ed6\u65b9\u306eF0\u30de\u30a6\u30b9\u304a\u3088\u3073\u305d\u306e\u5b50\u5b6b\u3067\u306f\u3001\u4e16\u4ee3\u9593\u306e\u907a\u4f1d\u5b50\u4f1d\u9054\u52b9\u7387\u306f\u4f4e\u4e0b\u3057\u3066\u3044\u305f\u3082\u306e\u306e\u3001\u751f\u307e\u308c\u305fF1\u30de\u30a6\u30b9\u306b\u306f\u7570\u5e38\u304c\u306a\u304b\u3063\u305f[48]\u3002\u307e\u305f\u3001double strand RNA\u3092\u767a\u73fe\u3057\u3066\u5185\u5728\u6027\u306bsiRNA\u3092\u7523\u751f\u3055\u305b\u308bpDECAP\u30b7\u30b9\u30c6\u30e0\u3092\u30c8\u30e9\u30f3\u30b9\u30b8\u30a7\u30cb\u30c3\u30af\u30de\u30a6\u30b9\u3068\u3057\u3066\u7528\u3044\u305f\u30ce\u30c3\u30af\u30c0\u30a6\u30f3\u30de\u30a6\u30b9[49]\u3067\u306f\u3001\u4e0d\u5b89\u95a2\u9023\u306e\u8a18\u61b6\u969c\u5bb3\u304c\u898b\u3089\u308c\u305f\u304c\u3001\u8133\u304a\u3088\u3073\u8178\u306a\u3069\u306e\u5168\u8eab\u81d3\u5668\u306b\u7570\u5e38\u306f\u306a\u304b\u3063\u305f\u3002\u4e00\u65b9\u3001\u30de\u30a6\u30b9\u80da\u4e00\u6b21\u795e\u7d4c\u7d30\u80de\u3067\u3053\u306e\u907a\u4f1d\u5b50\u3092\u30ce\u30c3\u30af\u30c0\u30a6\u30f3\u3057\u305f\u30e2\u30c7\u30eb\u3067\u306f\u3001\u30b9\u30d7\u30e9\u30a4\u30b7\u30f3\u30b0\u52b9\u7387\u306e\u4f4e\u4e0b\u304c\u660e\u3089\u304b\u306b\u306a\u308a\u3001\u305d\u306e\u7d50\u679c\u3001\u539f\u8178\u9665\u5165\u3084\u795e\u7d4c\u7ba1\u5f62\u6210\u306e\u30d1\u30bf\u30fc\u30f3\u306b\u7570\u5e38\u304c\u898b\u3089\u308c\u305f[12]\u3002\u3055\u3089\u306b\u3001Cre-LoxP\u30b7\u30b9\u30c6\u30e0\u306b\u304a\u3044\u3066\u3001Nestin-Cre\u3001Synapsin1-Cre\u3092\u7528\u3044\u305f\u30b3\u30f3\u30c7\u30a3\u30b7\u30e7\u30ca\u30eb\u30ce\u30c3\u30af\u30a2\u30a6\u30c8\u30de\u30a6\u30b9\u304c\u4f5c\u6210\u30fb\u5831\u544a\u3055\u308c\u3066\u3044\u308b[50][51]\u3002Nestin-Cre\u306b\u3088\u3063\u3066\u80ce\u5150\u671f\u8133\u5ba4\u4e0b\u5e2f\u306e\u795e\u7d4c\u5e79\u7d30\u80de\u304b\u3089PQBP1\u3092\u6b20\u640d\u3055\u305b\u308b\u3068\u5c0f\u982d\u75c7\u304c\u518d\u73fe\u3055\u308c\u3001\u3053\u308c\u306b\u5bfe\u3057\u3066AAV-PQBP1\u306b\u3088\u308b\u80ce\u5150\u907a\u4f1d\u5b50\u6cbb\u7642\u3092\u884c\u3046\u3068\u5c0f\u982d\u75c7\u304c\u6539\u5584\u3057\u3001\u51fa\u751f\u5f8c\u306e\u8a18\u61b6\u6a5f\u80fd\u969c\u5bb3\u3082\u6539\u5584\u3057\u305f[52]\u3002Synapsin1-Cre\u306b\u3088\u308b\u6210\u719f\u30cb\u30e5\u30fc\u30ed\u30f3\u304b\u3089\u306ePQBP1\u6b20\u640d\u3067\u306f\u3001\u5c0f\u982d\u75c7\u306f\u8d77\u3053\u3089\u306a\u3044\u3082\u306e\u306e\u3001\u30b7\u30ca\u30d7\u30b9\u7570\u5e38\u3084\u8a18\u61b6\u6a5f\u80fd\u969c\u5bb3\u304c\u898b\u3089\u308c\u305f[53]\u3002\u30b7\u30e7\u30a6\u30b8\u30e7\u30a6\u30d0\u30a8\u30e2\u30c7\u30eb\u3067\u306f\u3001\u30c8\u30e9\u30f3\u30b9\u30b8\u30a7\u30cb\u30c3\u30af\u904e\u5270\u767a\u73fe\u30e2\u30c7\u30eb[54]\u3068piggy Bac transposon\u306edPQBP1\u907a\u4f1d\u5b50\u306e5’\u4e0a\u6d41\u57df\u3078\u306e\u633f\u5165\u306b\u3088\u308b\u767a\u73fe\u4f4e\u4e0b\u30e2\u30c7\u30eb[55]\u304c\u5831\u544a\u3055\u308c\u3066\u3044\u308b\u3002\u904e\u5270\u767a\u73fe\u30e2\u30c7\u30eb\u3067\u306f\u9577\u671f\u8a18\u61b6\u969c\u5bb3\u3068\u6027\u884c\u52d5\u7570\u5e38\u304c\u89b3\u5bdf\u3055\u308c[56]\u3001\u4e00\u65b9\u3001\u767a\u73fe\u4f4e\u4e0b\u30e2\u30c7\u30eb\u3067\u306f\u72ec\u7279\u306e\u5b66\u7fd2\u969c\u5bb3\u304c\u8a8d\u3081\u3089\u308c\u305f[57]\u3002\u7dda\u866b\u306b\u306f\u3001\uff12\u7a2e\u985e\u306ePQBP1\u30db\u30e2\u30ed\u30b0\u304c\u5b58\u5728\u3059\u308b\u304c\u3001\u305d\u308c\u3089\u306e\u5909\u7570\u4f53\u3092\u6301\u3064\u7dda\u866b\u30e2\u30c7\u30eb\u3067\u306f\u8102\u8cea\u7570\u5e38\u304c\u793a\u5506\u3055\u308c\u3066\u3044\u308b[58]\u3002\u3053\u308c\u3089\u306e\u30e2\u30c7\u30eb\u52d5\u7269\u306e\u5b9f\u9a13\u7d50\u679c\u304b\u3089\u3001PQBP1\u3092\u9069\u5207\u306b\u6a5f\u80fd\u3055\u305b\u308b\u305f\u3081\u306b\u306f\u3001\u30bf\u30f3\u30d1\u30af\u8cea\u306e\u767a\u73fe\u91cf\u304c\u4e00\u5b9a\u306e\u7bc4\u56f2\u5185\u3067\u3042\u308b\u3053\u3068\u304c\u5fc5\u8981\u3067\u3042\u308b\u3068\u8003\u3048\u3089\u308c\u3066\u3044\u308b[59]\u3002\u7279\u306b\u3001\u30b7\u30e7\u30a6\u30b8\u30e7\u30a6\u30d0\u30a8\u306b\u304a\u3051\u308b\u7a2e\u3005\u306e\u30e2\u30c7\u30eb\u306e\u7d50\u679c\u304b\u3089\u3001PQBP1\u306e\u5168\u8eab\u7684\u904e\u5270\u767a\u73fe\u306f\u5bff\u547d\u7b49\u306b\u5f71\u97ff\u3059\u308b\u53ef\u80fd\u6027\u304c\u793a\u5506\u3055\u308c\u308b\u3082\u306e\u306e\u3001\u8133\u795e\u7d4c\u7cfb\u306b\u9650\u5b9a\u3057\u305f\u904e\u5270\u767a\u73fe\u3067\u306f\u5bff\u547d\u306b\u554f\u984c\u306f\u306a\u304f\u3001\u795e\u7d4c\u6a5f\u80fd\u3082\u6b63\u5e38\u306b\u7dad\u6301\u3067\u304d\u308b\u3068\u8003\u3048\u3089\u308c\u308b[60]\u3002\u76f8\u4e92\u4f5c\u7528[\u7de8\u96c6]PQBP1\u306f\u3001\u4ee5\u4e0b\u3068\u76f8\u4e92\u4f5c\u7528\u3059\u308b\u3053\u3068\u304c\u793a\u3055\u308c\u3066\u3044\u308b\u3002^ a b c GRCh38: Ensembl release 89: ENSG00000102103 – Ensembl, May 2017^ a b c GRCm38: Ensembl release 89: ENSMUSG00000031157 – Ensembl, May 2017^ Human PubMed Reference:^ Mouse PubMed Reference:^ \u201cPolar amino acid-rich sequences bind to polyglutamine tracts\u201d. Biochemical and Biophysical Research Communications 253 (1):  16\u201320. (December 1998). doi:10.1006\/bbrc.1998.9725. PMID\u00a09875212.\u00a0^ \u201cNovel truncating mutations in the polyglutamine tract binding protein 1 gene (PQBP1) cause Renpenning syndrome and X-linked mental retardation in another family with microcephaly\u201d. American Journal of Human Genetics 74 (4):  777\u201380. (April 2004). doi:10.1086\/383205. PMC\u00a01181956. PMID\u00a015024694. https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC1181956\/.\u00a0^ a b \u201cPQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival\u201d. Human Molecular Genetics 8 (6):  977\u201387. (June 1999). doi:10.1093\/hmg\/8.6.977. PMID\u00a010332029.\u00a0^ Imafuku, I.; Waragai, M.; Takeuchi, S.; Kanazawa, I.; Kawabata, M.; Mouradian, M. M.; Okazawa, H. (1998-12-09). \u201cPolar amino acid-rich sequences bind to polyglutamine tracts\u201d. Biochemical and Biophysical Research Communications 253 (1):  16\u201320. doi:10.1006\/bbrc.1998.9725. ISSN\u00a00006-291X. PMID\u00a09875212. https:\/\/pubmed.ncbi.nlm.nih.gov\/9875212.\u00a0^ Waragai, M.; Lammers, C. H.; Takeuchi, S.; Imafuku, I.; Udagawa, Y.; Kanazawa, I.; Kawabata, M.; Mouradian, M. M. et al. (1999-06). \u201cPQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival\u201d. Human Molecular Genetics 8 (6):  977\u2013987. doi:10.1093\/hmg\/8.6.977. ISSN\u00a00964-6906. PMID\u00a010332029. https:\/\/pubmed.ncbi.nlm.nih.gov\/10332029.\u00a0^ Waragai, M.; Lammers, C. H.; Takeuchi, S.; Imafuku, I.; Udagawa, Y.; Kanazawa, I.; Kawabata, M.; Mouradian, M. M. et al. (1999-06). \u201cPQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival\u201d. Human Molecular Genetics 8 (6):  977\u2013987. doi:10.1093\/hmg\/8.6.977. ISSN\u00a00964-6906. PMID\u00a010332029. https:\/\/pubmed.ncbi.nlm.nih.gov\/10332029.\u00a0^ Waragai, M.; Lammers, C. H.; Takeuchi, S.; Imafuku, I.; Udagawa, Y.; Kanazawa, I.; Kawabata, M.; Mouradian, M. M. et al. (1999-06). \u201cPQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival\u201d. Human Molecular Genetics 8 (6):  977\u2013987. doi:10.1093\/hmg\/8.6.977. ISSN\u00a00964-6906. PMID\u00a010332029. https:\/\/pubmed.ncbi.nlm.nih.gov\/10332029.\u00a0^ a b \u201cThe splicing factor PQBP1 regulates mesodermal and neural development through FGF signaling\u201d (\u82f1\u8a9e). Development 141 (19):  3740\u201351. (October 2014). doi:10.1242\/dev.106658. PMC\u00a04197583. PMID\u00a025209246. https:\/\/www.ncbi.nlm.nih.gov\/pmc\/articles\/PMC4197583\/.\u00a0^ Qi, Yunglong; Hoshino, Masataka; Wada, Yo-ichi; Marubuchi, Shigeki; Yoshimura, Natsue; Kanazawa, Ichiro; Shinomiya, Ken-ichi; Okazawa, Hitoshi (2005-09). \u201cPQBP-1 is expressed predominantly in the central nervous system during development\u201d. The European Journal of Neuroscience 22 (6):  1277\u20131286. doi:10.1111\/j.1460-9568.2005.04339.x. ISSN\u00a00953-816X. PMID\u00a016190883. https:\/\/pubmed.ncbi.nlm.nih.gov\/16190883.\u00a0^ Li, Chan; Ito, Hikaru; Fujita, Kyota; Shiwaku, Hiroki; Qi, Yunlong; Tagawa, Kazuhiko; Tamura, Takuya; Okazawa, Hitoshi (2013). \u201cSox2 transcriptionally regulates PQBP1, an intellectual disability-microcephaly causative gene, in neural stem progenitor cells\u201d. PloS One 8 (7):  e68627. doi:10.1371\/journal.pone.0068627. ISSN\u00a01932-6203. PMC\u00a03713010. PMID\u00a023874697. https:\/\/pubmed.ncbi.nlm.nih.gov\/23874697.\u00a0^ Okazawa, H.; Sudol, M.; Rich, T. (2001 Oct-Nov 1). \u201cPQBP-1 (Np\/PQ): a polyglutamine tract-binding and nuclear inclusion-forming protein\u201d. Brain Research Bulletin 56 (3-4):  273\u2013280. doi:10.1016\/s0361-9230(01)00579-2. ISSN\u00a00361-9230. PMID\u00a011719261. https:\/\/pubmed.ncbi.nlm.nih.gov\/11719261.\u00a0^ Waragai, M.; Lammers, C. H.; Takeuchi, S.; Imafuku, I.; Udagawa, Y.; Kanazawa, I.; Kawabata, M.; Mouradian, M. M. et al. (1999-06). \u201cPQBP-1, a novel polyglutamine tract-binding protein, inhibits transcription activation by Brn-2 and affects cell survival\u201d. Human Molecular Genetics 8 (6):  977\u2013987. doi:10.1093\/hmg\/8.6.977. ISSN\u00a00964-6906. PMID\u00a010332029. https:\/\/pubmed.ncbi.nlm.nih.gov\/10332029.\u00a0^ \u201cOMIM Entry: 300463 – POLYGLUTAMINE-BINDING PROTEIN 1; PQBP1\u201d. 2021\u5e7411\u670821\u65e5\u95b2\u89a7\u3002^ Takahashi, Masaki; Mizuguchi, Mineyuki; Shinoda, Hiroyuki; Aizawa, Tomoyasu; Demura, Makoto; Okazawa, Hitoshi; Kawano, Keiichi (2009-06). \u201cPolyglutamine tract binding protein-1 is an intrinsically unstructured protein\u201d. Biochimica Et Biophysica Acta 1794 (6):  936\u2013943. doi:10.1016\/j.bbapap.2009.03.001. ISSN\u00a00006-3002. PMID\u00a019303059. https:\/\/pubmed.ncbi.nlm.nih.gov\/19303059.\u00a0^ Rees, Martin; Gorba, Christian; de Chiara, Cesira; Bui, Tam T. T.; Garcia-Maya, Mitla; Drake, Alex F.; Okazawa, Hitoshi; Pastore, Annalisa et al. (2012-04-04). \u201cSolution model of the intrinsically disordered polyglutamine tract-binding protein-1\u201d. Biophysical Journal 102 (7):  1608\u20131616. doi:10.1016\/j.bpj.2012.02.047. ISSN\u00a01542-0086. PMC\u00a03318138. PMID\u00a022500761. https:\/\/pubmed.ncbi.nlm.nih.gov\/22500761.\u00a0^ Mizuguchi, Mineyuki; Okazawa, Hitoshi (2013). \u201c[Structural study of polyglutamine tract-binding protein 1\u201d]. Yakugaku Zasshi: Journal of the Pharmaceutical Society of Japan 133 (5):  519\u2013526. doi:10.1248\/yakushi.13-00001-2. ISSN\u00a01347-5231. PMID\u00a023649393. https:\/\/pubmed.ncbi.nlm.nih.gov\/23649393.\u00a0^ Waragai, M.; Junn, E.; Kajikawa, M.; Takeuchi, S.; Kanazawa, I.; Shibata, M.; Mouradian, M. M.; Okazawa, H. (2000-07-05). \u201cPQBP-1\/Npw38, a nuclear protein binding to the polyglutamine tract, interacts with U5-15kD\/dim1p via the carboxyl-terminal domain\u201d. Biochemical and Biophysical Research Communications 273 (2):  592\u2013595. doi:10.1006\/bbrc.2000.2992. ISSN\u00a00006-291X. PMID\u00a010873650. https:\/\/pubmed.ncbi.nlm.nih.gov\/10873650.\u00a0^ Zhang, Y.; Lindblom, T.; Chang, A.; Sudol, M.; Sluder, A. E.; Golemis, E. A. (2000-10-17). \u201cEvidence that dim1 associates with proteins involved in pre-mRNA splicing, and delineation of residues essential for dim1 interactions with hnRNP F and Npw38\/PQBP-1\u201d. Gene 257 (1):  33\u201343. doi:10.1016\/s0378-1119(00)00372-3. ISSN\u00a00378-1119. PMID\u00a011054566. https:\/\/pubmed.ncbi.nlm.nih.gov\/11054566.\u00a0^ Mizuguchi, Mineyuki; Obita, Takayuki; Serita, Tomohito; Kojima, Rieko; Nabeshima, Yuko; Okazawa, Hitoshi (2014-04-30). \u201cMutations in the PQBP1 gene prevent its interaction with the spliceosomal protein U5-15 kD\u201d. Nature Communications 5:  3822. doi:10.1038\/ncomms4822. ISSN\u00a02041-1723. PMID\u00a024781215. https:\/\/pubmed.ncbi.nlm.nih.gov\/24781215.\u00a0^ Wang, Qingqing; Moore, Michael J.; Adelmant, Guillaume; Marto, Jarrod A.; Silver, Pamela A. (2013-03-15). \u201cPQBP1, a factor linked to intellectual disability, affects alternative splicing associated with neurite outgrowth\u201d. Genes & Development 27 (6):  615\u2013626. doi:10.1101\/gad.212308.112. ISSN\u00a01549-5477. PMC\u00a03613609. PMID\u00a023512658. https:\/\/pubmed.ncbi.nlm.nih.gov\/23512658.\u00a0^ Okazawa, Hitoshi; Rich, Tina; Chang, Alex; Lin, Xi; Waragai, Masaaki; Kajikawa, Masunori; Enokido, Yasushi; Komuro, Akihiko et al. (2002-05-30). \u201cInteraction between mutant ataxin-1 and PQBP-1 affects transcription and cell death\u201d. Neuron 34 (5):  701\u2013713. doi:10.1016\/s0896-6273(02)00697-9. ISSN\u00a00896-6273. PMID\u00a012062018. https:\/\/pubmed.ncbi.nlm.nih.gov\/12062018.\u00a0^ Okazawa, H.; Sudol, M.; Rich, T. (2001 Oct-Nov 1). \u201cPQBP-1 (Np\/PQ): a polyglutamine tract-binding and nuclear inclusion-forming protein\u201d. Brain Research Bulletin 56 (3-4):  273\u2013280. doi:10.1016\/s0361-9230(01)00579-2. ISSN\u00a00361-9230. PMID\u00a011719261. https:\/\/pubmed.ncbi.nlm.nih.gov\/11719261.\u00a0^ a b Okazawa, Hitoshi; Rich, Tina; Chang, Alex; Lin, Xi; Waragai, Masaaki; Kajikawa, Masunori; Enokido, Yasushi; Komuro, Akihiko et al. (2002-05-30). \u201cInteraction between mutant ataxin-1 and PQBP-1 affects transcription and cell death\u201d. Neuron 34 (5):  701\u2013713. doi:10.1016\/s0896-6273(02)00697-9. ISSN\u00a00896-6273. PMID\u00a012062018. https:\/\/pubmed.ncbi.nlm.nih.gov\/12062018.\u00a0^ Kunde, S. A.; Musante, L.; Grimme, A.; Fischer, U.; M\u00fcller, E.; Wanker, E. E.; Kalscheuer, V. M. (2011-12-15). \u201cThe X-chromosome-linked intellectual disability protein PQBP1 is a component of neuronal RNA granules and regulates the appearance of stress granules\u201d. Human Molecular Genetics 20 (24):  4916\u20134931. doi:10.1093\/hmg\/ddr430. ISSN\u00a01460-2083. PMID\u00a021933836. https:\/\/pubmed.ncbi.nlm.nih.gov\/21933836.\u00a0^ Shen, Yuqian; Zhang, Zi Chao; Cheng, Shanshan; Liu, An; Zuo, Jian; Xia, Shuting; Liu, Xian; Liu, Wenhua et al. (2021-04-01). \u201cPQBP1 promotes translational elongation and regulates hippocampal mGluR-LTD by suppressing eEF2 phosphorylation\u201d. Molecular Cell 81 (7):  1425\u20131438.e10. doi:10.1016\/j.molcel.2021.01.032. ISSN\u00a01097-4164. PMID\u00a033662272. https:\/\/pubmed.ncbi.nlm.nih.gov\/33662272.\u00a0^ Ito, H.; Shiwaku, H.; Yoshida, C.; Homma, H.; Luo, H.; Chen, X.; Fujita, K.; Musante, L. et al. (2015-04). \u201cIn utero gene therapy rescues microcephaly caused by Pqbp1-hypofunction in neural stem progenitor cells\u201d. Molecular Psychiatry 20 (4):  459\u2013471. doi:10.1038\/mp.2014.69. ISSN\u00a01476-5578. PMC\u00a04378255. PMID\u00a025070536. https:\/\/pubmed.ncbi.nlm.nih.gov\/25070536.\u00a0^ Ito, H.; Shiwaku, H.; Yoshida, C.; Homma, H.; Luo, H.; Chen, X.; Fujita, K.; Musante, L. et al. 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ISSN\u00a01558-1497. PMID\u00a022901698. https:\/\/pubmed.ncbi.nlm.nih.gov\/22901698.\u00a0^ Tamura, Takuya; Sone, Masaki; Nakamura, Yoko; Shimamura, Teppei; Imoto, Seiya; Miyano, Satoru; Okazawa, Hitoshi (2013-01). \u201cA restricted level of PQBP1 is needed for the best longevity of Drosophila\u201d. Neurobiology of Aging 34 (1):  356.e11\u201320. doi:10.1016\/j.neurobiolaging.2012.07.015. ISSN\u00a01558-1497. PMID\u00a022901698. https:\/\/pubmed.ncbi.nlm.nih.gov\/22901698.\u00a0^ \u201cInteraction between mutant ataxin-1 and PQBP-1 affects transcription and cell death\u201d. Neuron 34 (5):  701\u201313. (May 2002). doi:10.1016\/s0896-6273(02)00697-9. PMID\u00a012062018.\u00a0^ a b \u201cEvidence that dim1 associates with proteins involved in pre-mRNA splicing, and delineation of residues essential for dim1 interactions with hnRNP F and Npw38\/PQBP-1\u201d. Gene 257 (1):  33\u201343. (October 2000). doi:10.1016\/s0378-1119(00)00372-3. PMID\u00a011054566.\u00a0^ \u201cAssociation of two nuclear proteins, Npw38 and NpwBP, via the interaction between the WW domain and a novel proline-rich motif containing glycine and arginine\u201d. The Journal of Biological Chemistry 274 (51):  36513\u20139. (December 1999). doi:10.1074\/jbc.274.51.36513. PMID\u00a010593949.\u00a0\u63a8\u85a6\u6587\u732e[\u7de8\u96c6]\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\/wiki7\/#breadcrumbitem","name":"Enzyklop\u00e4die"}},{"@type":"ListItem","position":2,"item":{"@id":"https:\/\/wiki.edu.vn\/jp\/wiki7\/2018\/09\/17\/%e3%83%9d%e3%83%aa%e3%82%b0%e3%83%ab%e3%82%bf%e3%83%9f%e3%83%b3%e7%b5%90%e5%90%88%e3%82%bf%e3%83%b3%e3%83%91%e3%82%af%e8%b3%aa-1-wikipedia\/#breadcrumbitem","name":"\u30dd\u30ea\u30b0\u30eb\u30bf\u30df\u30f3\u7d50\u5408\u30bf\u30f3\u30d1\u30af\u8cea-1 – Wikipedia"}}]}]