Êɂ^pN̕iǗ̕q@\ђ_onɂ鏬ẼXgX̖

<strong> pN</strong>
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emailF<a href="mailto:nishitoh@med.miyazaki-u.ac.jp">pN</a>
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̈Zr[, <strong>4</strong>, e009 (2015)@<a href="http://dx.doi.org/10.7875/leading.author.4.e009">DOI: 10.7875/leading.author.4.e009</a>
Hideki Nishitoh: <strong>The molecular mechanisms of endoplasmic reticulum quality control and the roles of unfolded protein response in central nervous system.</strong>

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<h2>v </h2>
@זEɋ@\ɂ́C`ɂꂽ^pN\Ƃ邱ƂKvsłDCwICIC`IȂ܂܂ȃXgXɂ萳\ƂĂȂsǂȃ^pNÊɒ~ς邱Ƃ͍זE̐ȋ@\̂܂ɂȂD̂悤Ȋ@IȏԂ邽߁CזEUPRƂ΂IȃXgXɂ鏬Êɂ^pN̕iǗ@\Cɂsǃ^pN͐@\𔭊DCÊɉߓx̕sǃ^pN~ςUPRł͑ΏȂȂƂC邢́CזEXgXɂUPRj]Ƃɂ̓A|g[VXUDُȍ\^pN̒~ςƂRz[Va̕q@\ƂāCẼXgXɂUזE@\̒ቺA|g[VX̊֗^ڂĂDCŋ߂ł́C܂܂ȑɂUPR̐IȖ炩ɂCUPR͐̂̋@\ɕK{̃XgXł邱Ƃ킩ĂD̃r[ł́CÊɂă^pN͂ǂ̂悤ɕiǗĂ̂C܂CẼXgX̖ɂāC_onɏœ_ڂD

<h2>͂߂</h2>
@זEVɍ^pN̑͐\ƂĂȂsǃ^pNł邪C̖1/3͖|̂̂ɃrL`-veA\[nɂ蕪<a href="#R1"><sup>1)</sup></a>DC|̓r̃|\[̖8͎ɂ\Ȃ炩̃IKlƌĂ<a href="#R2"><sup>2)</sup></a>CׂẴ^pN̖1/3͏Êʉ߂ƂĂDāC{\[̑͏E̖邢͂̋ߖTɂĖ|sĂCō^pN̂Ȃ̊sǃ^pNƂďÊɕׂĂƍlD^j̍זEɂčꂽ^pNі^pŃCÊɂč\`@\Iȃ^pNƂȂDCזE܂܂ȃXgXɂ炳sǃ^pNÊɒ~ςƏẼXgXƂԂɂDזE͂̃oX̗ꂽԂmP퐫ێ邽߂̃^pN̕iǗ@\Ƃđ傫2̎iƂi<a href="#F1">}1</a>jDЂƂ́CÊɑ}VK̃^pN̍}邱Ƃɂ菬Êɂ镉ׂyiłD̋@\ɂĂɊ^̂́CזȆŜɂ|̗}CсCÊɑ}^pNR[hmRNA𕪉RIDDiregulated IRE1-dependent decayjłD܂C^pÑgXRÊւ̑}ٓIɗ}CזEɂĂ𕪉邱Ƃɂ菬Êւ̂Ȃ镉ׂy\hIȕiǗ@\ipre-emptive quality controlFpQCjɂĂ񍐂Ă<a href="#R3"><sup>3,4)</sup></a>DЂƂ́Cłɍꂽsǃ^pNiłDȂ킿CẼVyATPpă^pNӂѐ܂肽ݐ\`邩C邢́C͂₻ꂪs\ȕsǃ^pNɂĂ͍זEɋtAĂ𕪉ERADiendoplasmic reticulum-associated degradationCE̊֘AjłD̂悤ȏÊɂ^pN̕iǗ@\͑čL`UPRiunfolded protein responsejƂ΂DƂ낪CUPRɂXgX̉\悤ȏẼXgXׂꂽꍇ^pN̕iǗ@\ቺꍇɂ̓A|g[VX̃VOiMDẼXgX͏E̖ɑ݂êɂ芴mC̃VOi͏Ê̊OւƓ`BD܂ŁCêẼXgXF镪q@\C̃GtFN^[уVOi`B̕q@\̑炩ɂĂD̃r[ł́CUPR̕q@\ƂƂɁĈ̖ЂƂƂāC_onɂUPR̐IȈӋ`Ƃ̔j]ɂ鎾Ƃ̊֌WɂčŐV̒m܂Ƃ߂D

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<h2>1DẼXgX̎e</h2>
@ҒœɂāCÊɂsǃ^pN̒~ς邢͏E̖\鎉̕ώCj[NȊ̋@\3ނ̎éCPERKCIRE1CATF6ɂFD͂ђʌ^̃^pNłCԂł͏E̓oɕqVyBiP/GRP78ĕs^ƂĈێĂDÊɂsǃ^pN̒~ςɂBiPêsǃ^pNւƈڍs邱ƂƂȂC̎ê͊ꉺɃVOi`BD
@PERK͍זEɃZ/XIjLi[[hCDẼXgXmPERK͓ʑ̉ɃIS}[ɂƂȂLi[[hCȃ_C\ω邱Ƃɂ芈^ɂȂ<a href="#R5"><sup>5)</sup></a>i<a href="#F2">}2</a>jD^PERK͊łeIF2ٓIɃ_D^jɂ|̑̓LbvˑIȖ|CȂ킿CMet-tRNACGTPCeIF2-eIF-eIFȂOҕ̂5f̖[ɃLbv\mRNAƖ|JnqeIF4FƂ̂̕ƌ邱ƂɂJnDeIF2̃_ɂOҕ̂̌`jQ邱ƂŃLbvˑIȖ|͗}CזȆŜɂ|̑x͒ቺCÊɑ}VK̃^pN̍<a href="#R6"><sup>6)</sup></a>DɂCÊւ̂Ȃ镉ׂ͌yDŁC_ꂽeIF2uORF\`q̖|tɑiD̓ȕq@\ɂ蔭̗Uꂽ]ʈqATF4́CA~m_ӂ̑i⊈_f̌yȂǂɂ`q̓]ʂU<a href="#R7"><sup>7)</sup></a>D̋@\͓IXgXiintegrated stress responsejƂ΂C_XgX_fXgXȂǂƂʂ鉞łDƂ낪CPERK-ATF4oH̓A|g[VX̑iɂ]ʈqCCHOPGADD153̔U邱ƂɂCẼXgX̋x邢͎ԂȂǂɉăA|g[VX̃VOiUD

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PF_D
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@IRE1͍זEɃLi[[hCRNasehC̗Iȍ\CMނɂIRE1IRE1݂DIRE1͂قڂׂĂ̑܂זEɔĂCIRE1̔͒ǌnȂǂɌ肳CƂɔtזEɂ郀`̎YɕK{ł<a href="#R8"><sup>8)</sup></a>DPERKƓlɁCIRE1ẼXgXmBiP̉𗣂̂̂ʑ̉CLi[[hCȃ_\ω邱ƂɂRNasehCDMނɂẮC^RNasehC͂̓ٓIȊł<em>XBP1</em> mRNA2JؒfC26̃CgXvCVOꂽ̂ؒfꂽGL\A<a href="#R9"><sup>9-11)</sup></a>i<a href="#F2">}2</a>jD<em>XBP1</em> mRNǍIȃXvCVO̓{\[ƋčꂽXBP1^pÑ|yv`hE̖ɃN[g邱Ƃɂ\ɂȂ<a href="#R12"><sup>12,13)</sup></a>DGL\A郊K[[ɂĂ͒słCŋ߁CRtcB񍐂ꂽ<a href="#R14"><sup>14)</sup></a>DXvCVOꂽ<em>XBP1</em> mRNA͓]ʈqXBP1sR[hĂCERADɊ֘A^pN_ҌyfȂǂ𔭌D܂CIS}[IRE1͕^pN▌^pNR[hmRNAؒf̔}D̋@\͂ɏqׂ悤RIDDƂ΂<a href="#R15"><sup>15,16)</sup></a>CẼXgXɂUA|g[VXƂ̊֘AĂ<a href="#R17"><sup>17)</sup></a>DIɊꂽIRE1mRNÂ݂Ȃ炸CmiR-17CmiR-34aCmiR-96CmiR-125bIIɐؒf<a href="#R18"><sup>18)</sup></a>D̃}CNRNA̓JXp[[2̔̐ɂĂČʁCJXp[[oH̊ăA|g[VX𑣐iBcl-2t@~[^pN~gRhAւƈڍs邱ƂɂA|g[VXUDCRNaseɔˑIIRE1̋@\ƂāCNF-BoHJNKoH̊DLi[[hC̊ɈˑăA_v^[^pNTRAF2IRE1ւƃN[g<a href="#R19"><sup>19)</sup></a>CASK1JNKoHA|g[VXU<a href="#R20"><sup>20)</sup></a>DǉɂĂIRE1TRAF2Ƃ̌IKK̃_p65Tujbg̕iNF-BoH<a href="#R21"><sup>21)</sup></a>D̂悤ɁCIRE1͏ẼXgX̏󋵂ɉĂ܂܂ȃVOi`BoH邱ƂɂCƋ@\ɂ̓A|g[VXƂlȍזẺ^SĂCXgX̋ƒɉLi[[hCRNasehC̊ǂ̂悤ɔF̂[D
@ATF6C[E̓oނђʌ^̃^pNŁCN[bZIPhC^̓]ʈqłDҒœɂATF6ATF6݂Cꂼꂪ㏞ɋ@\<a href="#R22"><sup>22)</sup></a>DATF6͏ẼXgXɈˑăSŴւƗACɑ݂site-1veA[[site-2veA[[ɂ薌̓ɂĐؒf<a href="#R23"><sup>23)</sup></a>i<a href="#F2">}2</a>jDؒfꂽN[̒fЂ͊jւƈڍsCBiPȂǂ̕qVy̓]ʂU邱Ƃɂ菬Êɂ^pN̍\̌`コD܂ŁCATF6Ɨގ̍\5ނOASISt@~[^pNCOASIS/CREB3L1CBBF2H7/CREB3L2CCREBH/CREB3L3CAIbZIP/CREB3L4/Tisp40/CREB4CLuman/LZiP/CREB3肳ĂDCSŴɂĖ̓ɂĐؒfN[̒fЂ]ʈqƂċ@\D͍זEɓٓIȔ̂COASIS͍זE<a href="#R24"><sup>24)</sup></a>CAXgTCg<a href="#R25"><sup>25,26)</sup></a>C咰̔tזE<a href="#R27"><sup>27)</sup></a>CBBF2H7͓זE<a href="#R28"><sup>28)</sup></a>C]<a href="#R29"><sup>29)</sup></a>CCREBH͊̑<a href="#R30"><sup>30)</sup></a>CCREB4͐<a href="#R31"><sup>31,32)</sup></a> ȂǂɔC̕@\ɂďdvȖ͂ĂDŋ߁COASISt@~[̐Vȋ@\ƂāCBBF2H7C[̒fЂזE̊Oւƕ傳ꔭɂwbWzbOVOi`BoHזȆBɊ֗^邱Ƃ񍐂ꂽ<a href="#R28"><sup>28)</sup></a>D̂悤ɁCATF6t@~[̓SŴɂĖ̓ł̐ؒfƂj[Nȕq@\ɂ芈邪C̑lȐwIȈӋ`Ɋւ邳Ȃ͂҂D

<h2>2DÊɂ^pN̕iǗ@\</h2>
@זEXgXۂɏÊɂă^pN̕iێ邽ߍŏɂƂ헪́CPERKɂeIF2̃_זȆŜɂ|̗}łDCIȖ|̗}͍זE̐ɂƂssȂ߁C_eIF2̉ɂĔ̗U]ʈqATF4ɂGADD34CPP1ƂƂeIF2E_<a href="#R33"><sup>33)</sup></a>i<a href="#F2">}2</a>jD̃̕tB[hobN@\ɂߓIȖ|̗}̂̏ẼVyERADɊ֘A^pN̗̔UȂǂ\ɂDC|̍ۂɏÊɑ}^pNɂ͏ẼVyȂǂ̂قɂ̕^pNDVOiz^pN͒Ԃł͏Êɑ}邪CẼXgX̂Ƃł̓^pN̕iǗɕsvȃ^pNȂׂȂ悤ɂ邽߁CÊւ̑}ٓIɑjQčזEɂĒڂɕ\hIȕiǗ@\݂<a href="#R3"><sup>3,4)</sup></a>i<a href="#F1">}1</a>jD̗\hIȕiǗ@\̔j]vIa̕aԂȂǂƊ֘A邱Ƃ񍐂ĂC_onɂ^pN̕iǗэזE̋@\̈ێɕK{ł邱ƂĂ邪<a href="#R34"><sup>34)</sup></a>Cɂ镪q@\ɂĂ͌_ł͕słD
@UPRɂ3̌oHCPERK-eIF2-ATF4oHCIRE1-XBP1oHCATF6oH́CBiPȂǂ̏ẼVŷقɂC_ҌyfCN`^pNCERADɊ֘A^pNȂǁCÊɂ^pN̕iǗɂ邳܂܂ȃ^pN̔UDÊɑ}ꂽ^pŃCVOiz̐ؒfC̕tɂЂÂCWXtBȟ`Ȃǂ̖|CsDÊ͍זEɔ׎_ԂłĈƂ̓WXtBh\̌`ɏdvłD̏Ê̓̊Ɉێ邽߁CEro1PDIȂǖ20ނ̎_Ҍyf̗̔ʂ⑊ݍp䂳Ă<a href="#R35"><sup>35)</sup></a>D܂C^pN̕iǗɂ̓JlLVуJeBLN`^̕qVyƂĎ_ِyfERp57Ƌč\̌`ɂ<a href="#R36"><sup>36,37)</sup></a>D̂悤ɁCÊł́CqVyC_ҌyfCN`^pNȂǍ\̌`ɂ鑽̃^pNC܂܂Ȏނ̕sǃ^pN̐܂肽ُ݂̈FCqVyATPɈˑIȃVy_ِ𗘗pĐ̍\`ĂDC̍\̌`ǂĂ܂i܂ȂꍇC`qψقɂA~m_ψقꍇȂǂɂ͕sǃ^pN͂̕oHłERADoHւƑ荞܂D̍\̌`̕ʂS^pNƂẮCҌyfERdj5 <a href="#R38"><sup>38-40)</sup></a> ⃌N`^pNEDEM <a href="#R41"><sup>41)</sup></a> ȂǂdvȖ͂i<a href="#F3">}3</a>jD

<a name="F3"></a><div id="fig3-caption-text" style="display: none;"><strong>}3@ERADMAM̍\^pN</strong>
IP3FCmVg[1,4,5gX_D
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@ÊɃ^pN̕n݂͑Ȃ߁C̍\̌`̕s\ȕsǃ^pNERADoHɂ菬ÊזEւƋtAD̍ۂɕsǃ^pNʉ߂郌ggXRɂẮCSec61gXRłƂ<a href="#R42"><sup>42)</sup></a>CDerlint@~[^pN<a href="#R43"><sup>43,44)</sup></a> ⃆rL`K[[HRD1 <a href="#R45"><sup>45)</sup></a> ȂERADɊ֘A^pNE`ĂƂC邢́CdqɂזE̕\ʂ̊ώ@ɂgXR̂قɂ͍E炵\ώ@ȂƂtAE݂͑Ȃ̂ł͂ȂƂ<a href="#R46"><sup>46)</sup></a>C̎Ԃ͂܂ׂƂĂDזEɕoꂽsǃ^pN̓yv`h:<em>N</em>-OJi[[ɂ蓜ꂽ̂<a href="#R47"><sup>47)</sup></a>CrL`-veA\[nɂ蕪<a href="#R48"><sup>48)</sup></a>DERADɂ蕪^pŃC\Ɉُ̂̈ɉERAD-CiזEjCERAD-LiÉjCERAD-MiE̖jɑʂ<a href="#R49"><sup>49)</sup></a>DoyɂẮCERAD-Cɂ̓rL`K[[Doa10CERAD-Lɂ̓rL`K[[Hrd1pCERAD-MɂDoa10Hrd1p̗҂֗^邱Ƃ`wIɎĂDCMނɂẮCHRD1/SynoviolinioyHrd1pjCgp78/AMFR/RNF45CRMA1/RNF5CTEB4/MARCH6ioyDoa10jCTMEM129CRNF103/Kf-1CRNF126CRNF138/Trc8CRNF170CRFP2/TRIM13ȂǁC悻10ނ̃rL`K[[֗^邪<a href="#R50"><sup>50)</sup></a>Cꂼꂼ̖̈ႢɂĂ͂ɏڍׂȌKvłDrL`ꂽ^pNDerlint@~[^pNɌp97ioyCdc48jƂ̕qłNpl4Ufd1Ƃ̂̕Cp97ATPɈˑăveA\[ւƗA<a href="#R43"><sup>43,51)</sup></a>D̍ہC^pN̑ä悪זEŋÏW̕܂邱ƂȂ悤CVyl̊Bag6äƌ邱ƂɂIȕɊ^Ă<a href="#R52"><sup>52)</sup></a>i<a href="#F3">}3</a>jD̂悤ȈA̋@\ɂsǃ^pN͏ÊזEɂċÏW邱ƂȂX[YɏCזE͏ẼXgXĐ@\邱Ƃ\ɂȂDCȂ炩̌ɂ肱̕nj]ƍזE̋@\̒ቺזEɂȂC܂܂Ȏ̌ɂȂD

<h2>3DUPRƃ~gRhA</h2>
@Êɂ̓^pN̍ȑeʏÊ⎉̍̏Ƃċ@\銊ʏÊ͂߁C܂܂ȃTuhC݂DÊ͊j͂܂זȆŜɍL\łCق̃IKlƐڐG镪q@\ڐG_̌`鐶IȈӋ`ڂĂDƂɁC~gRhAƐڐGĂ̈MAMimitochondria-associated membranejƂ΂CI[gt@S\[̌`<a href="#R53"><sup>53)</sup></a>CсCCt}\[̌`<a href="#R54"><sup>54)</sup></a> ɂ钆SIȗ̈łDMAM͕̃^pNɂ2̃IKln邱Ƃɂ`i<a href="#F3">}3</a>jDCa<sup>2+</sup>̗⎉̋ёӂȂǁCזE̋@\̈ێɂďdvȖ͂Dŋ߁CPERKMAMɋǍ݂邱ƂoCMAM̌`у~gRhAɂCa<sup>2+</sup>P퐫̈ێɊ^Ă邱Ƃ炩ɂꂽC̏ڍׂȕq@\͕sł<a href="#R55"><sup>55,56)</sup></a>D́CUPRMAMɂ鐶IȖт̕q@\̉𖾂҂D

<h2>4D̕ωUPR</h2>
@sǃ^pN̒~ςłȂCזE̖̍P퐫̔j]ẼXgXU܂܂ȎɊ֗^邱Ƃ炩ɂĂD̖OaƕsOaƂ̃oX邱ƂɂPERKIRE1͑IIɊ<a href="#R57"><sup>57-59)</sup></a>D̍\̕ωɂUPR̊͂܂قȂ镪q@\ɂDE̓ohCPERKIRE1̖Oaɂ芈ꂽƂ<a href="#R58"><sup>58)</sup></a>C̕ω͖ђʃhC̋ߖTɂĊmĂƗ\z邪Cڍׂȕq@\͕słD܂C̖OaɂUPRVOiƕsǃ^pN̒~ςɂUPRVOiƂ͎IɈقȂ̂Cł΁C̕q@\ɊւĂ[Db̑ӂƑӐƂ̊֘AɁCCɎUPR̊֘AɂĂ̌iނƂ҂D

<h2>5DẼXgXɂUA|g[VX</h2>
@זEUPRɂłȂ悤ȉߓx̏ẼXgXꍇC킳ɂ͌̂̈ێ̂߈ُȋ@\𔭊Ȃ悤ɍזE͎玀ɂD̂Ƃ̍זÉCÊɂsǃ^pN̒~ςɂЂÂ~gRhǍ`ԂɕωNCVgN<em>c</em>̕oJXp[[3̊Ȃǂώ@CŏIITUNELzj̒fЉUD̍זE̓JXp[[̑jQ܂ɂ}邱ƂCẼXgXɂUזE̓A|g[VXłƂDȉCẼXgXɂU邨ȃA|g[VXoHɂċLqi<a href="#F4">}4</a>jD

<a name="F4"></a><div id="fig4-caption-text" style="display: none;"><strong>}4@ẼXgXɂUA|g[VXɊ֘AUPR</strong>
PF_CIP3FCmVg[1,4,5gX_D
<a href="http://leading.lifesciencedb.jp/wordpress/wp-content/uploads/2015/06/Nishitoh-4.e009-Fig.4.jpg" target="_blank">[Download]</a></div>[hs_figure id=4&amp;image=/wordpress/wp-content/uploads/2015/06/Nishitoh-4.e009-Fig.4.png&amp;caption=fig4-caption-text]

@ẼXgXɂUA|g[VXɊ֘A^pNƂčŏɕ񍐂ꂽ̂CHOPłDCHOPPERK-ATF4oHATF6oH̉ɂĔ̗U]ʈq̂ЂƂŁCWIƂȂ`q̎YƂẮCGADD34CEro1CDR5 <a href="#R60"><sup>60)</sup></a>CTrb3 <a href="#R61"><sup>61)</sup></a>CBim <a href="#R62"><sup>62)</sup></a>CPUMA <a href="#R63"><sup>63)</sup></a> ȂǂC̑A|g[VXɊ֘A@\DCHOPזEGADD34זEł͏ẼXgXɈˑIȃA|g[VX}邱Ƃ<a href="#R64"><sup>64-66)</sup></a>ČoH̏dv͖炩łD̂قɂCHOP̕WI͑񍐂ĂCXgXɈˑIC܂CזEɓٓIȈ`qăA|g[VXUĂƍl<a href="#R67"><sup>67)</sup></a>D
@ẼXgXɂUA|g[VXɊ֗^JXp[[́CJXp[[2CJXp[[3CJXp[[4CJXp[[7CJXp[[8CJXp[[9CJXp[[12łDŏɒڂꂽ̂̓}EX̃JXp[[12<a href="#R68"><sup>68)</sup></a>Cqgɂ邻̋@\IȃzO̓JXp[[4ł<a href="#R69"><sup>69)</sup></a>DE̖̋ߖTɋǍ݂CẼXgXɈˑIɐؒfĊ^ɂȂזEւƈڍsDJXp[[12זEł͏ẼXgXɈˑIȃA|g[VXɗ}ĂC̊̋@\́CÊCmVg[1,4,5gX_ê邢̓AmWêĕoꂽCa<sup>2+</sup>ɈˑIȊJpCIRE1-TRAF2̂ɃJXp[[12N[g邱Ƃɂ<a href="#R70"><sup>70,71)</sup></a>D^JXp[[12BID̐ؒfу~gRhÃVgN<em>c</em>̕oɂƂȂJXp[[9DJXp[[2уJXp[[8ɂĂlɁCBID̐ؒfăJXp[[3уJXp[[7̊ɈˑăA|g[VXU<a href="#R72"><sup>72,73)</sup></a>DJXp[[2ɂẮCɏqׂƂCIRE1ɈˑIȂmiRNA̐ؒfɂ蔭UƂj[Nȕq@\񍐂ĂDCJXp[[4̓JXp[[9𒼐ڂɐؒf<a href="#R74"><sup>74)</sup></a>D̂悤ɁC̃JXp[[t@~[ẼXgXɂUA|g[VXɊ֗^Ă邪C܂ł́C^vVKMcjJ}CVȂǖ܂ɂUꂽẼXgXɂ錤ĆCIȋǖʂł̏ẼXgXɂUꂽA|g[VXɂɂĂ̌dvłD
@A|g[VX𑣐iBcl-2t@~[^pNłBaxBak̓dזEɂẮCO̎hȂǂɂUA|g[VXƓlɁCẼXgXɂUA|g[VX}DBaxBaḱC̍p@Ƃă~gRhÂ݂Ȃ炸CE̖ɂCa<sup>2+</sup>P퐫ɂڂɊ֗^<a href="#R75"><sup>75)</sup></a>DBaxCCmVg[1,4,5gX_éCSec61gXRėoCa<sup>2+</sup>ZJhbZW[Ƃă~gRhAɍpA|g[VX̃VOi`BD]ʈqƂĒmRIP140͊j̊OւƈڍsďẼ̖CmVg[1,4,5gX_êƒڂɌ邱Ƃɂ菬ÊCa<sup>2+</sup>̕oɂƂȂA|g[VX𕉂ɐ䂷<a href="#R76"><sup>76)</sup></a>DBH-3̈悾Bcl-2t@~[^pNƂẮCPUMANOXAẼXgXɈˑIp53UCA|g[VX̎s^pNƂċ@\D
@IRE1̉ɂA|g[VX̗UɂẮCJXp[[̂قɃLi[[hCɈˑIASK1-JNKoHi<a href="#F4">}4</a>jDCIɊIRE1͂RNaseِ̓ቺE̖̋ߖTɑ݂鑽mRNAؒf<a href="#R17"><sup>17)</sup></a>D̕WIɂ́C^pN̂قqVyȂǃ^pN̕iǗɕKvȃ^pNmRNA܂܂ČʁCÊɂiǗ@\ቺA|g[VXUD

<h2>6D_oϐɂ鏬ẼXgX̊֗^</h2>
@̐_oϐɂĂ͎ɓٓIȈُ^pNזE邢͍זEOɒ~ςC_o@\̒ቺƂɂ̓j[̕ϐЂND̂悤Ȉُ^pN̒~ςɂẼXgX֗^D
@AcnC}[å҂ɂ͔]Ɉُȃ^pN̒~ϔ݂ĈЂƂA~Ch^pNvȐƂזEO̒łDAcnC}[ȃ͌ǔł邪ꕔ͉ƑłČ`qƂăA~ChÕ^pNCvZj1CvZj2R[h`qmĂD̈`qψقɂĂA~Ch^pN̎Y㏸邱ƂȂǂCA~Ch^pN̒~ς̓AcnC}[a̔ǂɊ֗^ƍlDA~Ch^pN̓A~ChÕ^pNZN^[[уZN^[[ivZj1̐ؒf^pNjɂؒfזE̊Oɕ傳40`42A~m_cȂyv`hŁC_oŐ𔭊j[̕ϐЂND|{ꂽj[ɂăA~Ch^pN̓YɂזEώ@邪C̍ۂɁCJXp[[12уJXp[[4ČזEɂĂ̓A~Ch^pNɂUj[̍זE͗}<a href="#R68"><sup>68,69)</sup></a>DזE̊ÕA~Ch^pNǂ̂悤ɂďẼXgXU邩CɁCAcnC}[aɂďẼXgXזEЂNƂ̔ǂɂȂĂ̂͋c_cƂłDCaԃfɂXBP1̔ɂA~Ch^pNɈˑIɗUj[̕ϐ}邱Ƃ<a href="#R77"><sup>77)</sup></a>Ĉق̕񍐂ɂA~Ch^pN̐_oŐUPR֗^邱ƂĂ邱ƂCn̕WIƂẲ\i<a href="#F5">}5</a>jD

<a name="F5"></a><div id="fig5-caption-text" style="display: none;"><strong>}5@_onɂUPR̖</strong>
<a href="http://leading.lifesciencedb.jp/wordpress/wp-content/uploads/2015/06/Nishitoh-4.e009-Fig.5.jpg" target="_blank">[Download]</a></div>[hs_figure id=5&amp;image=/wordpress/wp-content/uploads/2015/06/Nishitoh-4.e009-Fig.5.png&amp;caption=fig5-caption-text]

@p[L\å҂̒]jɂĂ̓j[̒EF߂Dǔ̃p[L\å҂ɂĎcj[ɂLewŷƂ΂镕̂ώ@CLewŷ̂Ȑ̓VkNCłDVkNC͉Ƒp[L\ǎ`q̎YƂĒmCPC12זEɂăVkNCψّ̂𔭌ƃveA\[̗}CVgN<em>c</em>̕oCJXp[[3уJXp[[9̊ɂ킦CẼXgXуJXp[[12̊ώ@<a href="#R78"><sup>78)</sup></a>DŁCẼXgXɈˑĊ]ʈqATF4ɂC͂p[L\ǎ`q̎YƂĒmParkin̔UזE}<a href="#R79"><sup>79)</sup></a>D1-`-4-tFj-1,2,3,6-egqhsW̔]ւ̓^ɂ閝p[L\ã}EXfɂẮCATF6CHOP̔U<a href="#R80"><sup>80)</sup></a>DC^XBP1s̈`q̓}EXɂăp[L\ȁǏP<a href="#R81"><sup>81)</sup></a>D̂悤ɁCp[L\afɂđUPR֘A`q̏dvĂCn̕WIƂĒڂĂi<a href="#F5">}5</a>jDCẼXgXЂN镪q@\ɂĂ͊_f̊֗^ȂǂĂ邪sȓ_D܂CẼXgX̃h[p~j[̃h[p~\ɉeĂ̂C܂Cj[̕ϐЂNĂ̂CC𖾂ׂ_͑cĂC܂܂ɑ݂p[L\afɋʂ镪q@\̉𖾂KvłD
@|O^~a͈`qɂăO^~R[hCAGԂُɐL邱Ƃɂ|O^~^pN邱ƂɋND|O^~^pN̓j[ɕ̂`_o@\̏Qj[̕ϐЂNDn`gaCҐ؈ޏkǁCMachado-JosephaȂǂɂđ̌`q肳ĂD|O^~^pN̈`qւ̉e₳܂܂ȃ^pNƂ̌ɂ_oŐ񍐂Ă邪ĈЂƂCUPS̑jQƂɂƂȂrL`^pN̒~ςɂj[̕ϐł<a href="#R82"><sup>82-84)</sup></a>i<a href="#F5">}5</a>jDj[ɂă|O^~^pN̒~ςɂveA\[̗}͏ẼXgXU邪<a href="#R20"><sup>20)</sup></a>C̕q@\ERAD̗}ɂƍlDUPRƂ̊֘AɂẮCXBP1ɂ|O^~^pN̓Ő̌y<a href="#R85"><sup>85)</sup></a>C΂ɁCPERK-eIF2oH̕KvĂ<a href="#R86"><sup>86)</sup></a>D|O^~^pNŐ𔭊邽߂ɕKvȃ^pNƂăVEWEoGp`wIѐwIȌ^pN̒Tɂ蓯肳ꂽ̂p97ł<a href="#R87"><sup>87,88)</sup></a>DŁC|O^~^pNp97ɌERAD}邱ƂɂCẼXgXɂU_oŐƂ񍐂Ă<a href="#R89"><sup>89,90)</sup></a>DCp97ɂĂERAD̂قɂ܂܂ȖmĂC̈ƂāC|O^~^pNƂ̌jɂDNAC̋@\jQ邱Ƃ<a href="#R91"><sup>91)</sup></a>DāCp97|O^~aɂǂ̂悤ɂ̂ɂĂ͂܂ׂƂĂD|O^~^pN̕ɂ̓I[gt@W[֗^邱ƂCזEɒ~ς|O^~^pNrL`-veA\[nłȂ܂܂ȕoHɉeʁCÊׂ̕債j[̐Ǝ㐫ɂĂ邱ƂlD̂悤ȃ^pN̕\̒ቺɂƂȂẼXgX̗Uɂ|O^~a̔ǋ@\ɂĂ͖𖾂ȓ_cĂ邪CfɂďẼXgX̌ỷ񕜂ɂȂ邱Ƃ񍐂Ă邱ƂCUPR͑n̕qWIƂĊ҂D
@؈ޏkdǂ͉^j[IIɒE邱ƂɂNdĂȉ^@\Q̎łD͌ǔł邪10ƑŁČƂȂ`qψق̖20̓X[p[ILVhWX^[[1R[h<em>SOD1</em>`qɑ݂C܂ł130ȏ̈`qψق񍐂ĂDψٌ^SOD1͐VȍזEŐl邱Ƃɂ^j[̕ϐUDveA\[̒ቺ͕ψٌ^SOD1̍זEŐ𑝋邱Ƃ<a href="#R92"><sup>92)</sup></a>CӔɔǂ؈ޏkdǂ̕aԋ@\ƂĉȂǂɂ^pN̕\̒ቺƕψٌ^SOD1̒~ςт̓Ő֗^Ɨ\zĎO[vCψٌ^SOD1ɂ_oŐƂďẼXgX̊֗^񍐂ĂDזEɒ~ςψٌ^SOD1Êɕsǃ^pN~ς镪q@\́CERAD֘A^pNłDerlin-1Ƃ̓ٓIȌɂDψٌ^SOD1Derlin-1Ƃ̌ERADɂ蕪^pN̋tAjQCʓIɁCÊɕsǃ^pN~ςi<a href="#F5">}5</a>jD̂悤ɂĂЂNꂽ^j[т̎͂̍זEɂ鏬ẼXgX́CASK1-JNKoH̊CJXp[[̊CCHOP̔U邱ƂɂC^j[̕ϐ邢͍זEЂN<a href="#R93"><sup>93-95)</sup></a>Dŋ߁C؈ޏkdǂɂ^j[̓ٓIȐƎ㐫Ɋւ镪q@\ɂĕ񍐂ꂽ<a href="#R96"><sup>96)</sup></a>DQ₷ҐOp̉^j[ɂĂBiP̃RVyłSIL1̔ቺĂĈ߁CÊɂ^pN̕iǗɂJeBLCDerlin-1CRbx1Ȃǂ̔ቺ邽ߏẼXgXɑ΂ƎɂȂD́CقڂׂĂ̍זEŔSOD1̈`qψقȂ^j[ٓIɏQ邩C̕q@\͂߂Ď̂łD؈ޏkdǂƏẼXgXƂ̊֌WɂĂSOD1̈`qψقłȂTDP-43̈`qψقɂĂ񍐂Ă<a href="#R97"><sup>97)</sup></a>CƑ݂̂Ȃ炸ǔ܂߂ׂĂ̋؈ޏkdǂɋʂ镪q@\ƂĕaԂɊ֘A\D́CUPRWIƂᕪq̒Tѕaԃfɂ錟҂D
@ȑOCXgXђ_fXgX̓j[ɂďÊ̋@\邵Q邱Ƃ`ԊwIɊώ@Ă<a href="#R98"><sup>98)</sup></a>DƂɁCCnCA1̈̃j[ɂẮCߐ̔]̂̂E̖ɂ^pN̍jQꏬEA~邱Ƃɂ菬Êɕsǃ^pN~ςg傷D̂悤ȋXgXђ_fXgXɂ鏬Ê̋@\̒ቺ͒_ȍQɂȂDCHOPmbNAEg}EXł͒_fɂQ̂̂̃j[̕ϐy<a href="#R99"><sup>99)</sup></a>C΂ɁCATF6mbNAEg}EXł̓OAזȆƂȂj[̕ϐ<a href="#R100"><sup>100)</sup></a>Dyߐ̔]ɂ肠炩UPRUĂBiPȂǂ̕qVy̔𑝑傳邱ƂɂĈ̂ɋN傫ȐNPɑ΂j[̑ϐ߂邱Ƃ\łD܂Čyߐ̔]ɂI[gt@W[̊܂C2Iȋɂ鏬ẼXgXׂ̕y<a href="#R101"><sup>101)</sup></a>i<a href="#F5">}5</a>jD̂悤ȂƂC]̑gDɂăI[gt@W[ERAD̗̕n@\邱Ƃɂ_o@\̍P퐫ۂĂƍlD

<h2>7D얞XgXɂUPR̒_onɂ</h2>
@얞ѓAa̕aԂ̋@\́C̑XȂǑ̋@\ُ̈ɋN邾łȂC_onSg֔Myv`hzӐn̔j]֗^邱ƂڂĂDӂɂ钆_onłUPRɊւĂ͎ɂĂ̏dvĂi<a href="#F5">}5</a>jD얞ⓜAał́C_oɖOH̃VOi𔭂얞}zł郌v`CXɑ΂R傷邪C̕q@\͕słD̃vIsImR`Yj[ɂẮCbHׂ̕ɂXBP1s̔U邱Ƃɂ背v`RуCXRɘaDɁCvIsImR`Yj[疖̊̑gDւƔMזEɔ񎩗XBP1sVOiē̑ӂƃCX󐫂񕜂<a href="#R102"><sup>102)</sup></a>DĈ悤Ȓ_onɂUPRЂƂєj]ƑSg̑ӈُɂȂC얞邢͓AȁԂ邱ƂɂȂ<a href="#R103"><sup>103)</sup></a>D܂CɏqׂMAM̍\^pN̂ЂƂłMFN2̃vIsImR`Yj[ɂ錇́CẼXgX̑ɂƂȂv`Rɂ얞܂˂<a href="#R104"><sup>104)</sup></a>D̂悤ɁC_oƂɎ̃vIsImR`Yj[ɂ鏬Ê̍P퐫̔j]͑Sg̑ӂ̏QɂȂ邱ƂCUPR̍זEɔ񎩗̃VOi`B̏dvڂĂDC얞ⓜAa܂ޑӐɂ鏬ẼXgX̊֗^ɂĂɖ炩ɂł낤D

<h2></h2>
@Êɂ^pN̕iǗ@\̉𖾂́C킪̑̌҂̍vɂƂ낪傫D̃r[łƂ肠ȊOɂClȕq@\𖾂ĂD̈ŁCggXR̎ԁCIRE1ɂXBP1̓ٓIȃXvCVORIDDɂ镝LRNA̐ؒf̕q@\̈ႢCATF6t@~[̖C\hIȕiǗ̕q@\ȂǁC܂܂sȓ_cĂD܂C]͖̌܂ɂUUPŘłCߔŃĈɂ鐶Iȏ̂Ƃł̃XgXɊւ錤i߂ĂD̂悤ȐIȏẼXgXƂ̔j]ɂ鎾̕q@\̉𖾂ɐiW邱ƂɂCɂċ̓IȕqWIɑ΂nɂȂ邱Ƃ҂D

<h2> </h2>
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</ol>
<div class="au-profile">
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<span class="author"> pNiHideki Nishitohj</span>
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</div>
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