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iww@wXoCITCGX ̖hwj
emailF<a href="mailto:yasutomo@tokushima-u.ac.jp">FN</a>
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̈Zr[, <strong>3</strong>, e013 (2014)@<a href="http://dx.doi.org/10.7875/leading.author.3.e013">DOI: 10.7875/leading.author.3.e013</a>
Koji Yasutomo: <strong>Molecular basis of autoinflammatory disorders.</strong>

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<h2>v </h2>
@ȍRɉ郊p̔ǂ̌ƂȂ鎩ȖƉuÂƉuaƂĒmĂDƑΔ䂳鎾ƂāCȉǐƂ΂鎾Ƒ̉ǐɂ錴`q̌mĂDȉǂƂ́CȖƉu▾炩Ȋ݂Ȃɂ炸ǂUaԂłCɕL̊ՂɂȂƍlDŋ߂̃Qm͎@̔WɂCȉǐ̕aԂU`qɂĉ𖾂i݂CNLRP3ɑ\NODlẽt@~[̈`qُɂ킦Cŋ߁CƉuveA\[̍\^pN̈`qُɂ鎩ȉǐ񍐂ꂽD̈`qPq̎ɂƂǂ܂炸q̎ɂ֗^邱Ƃ񍐂CCȉǂɊ֗^鎾͂܂܂炩ɂȂƗ\DƕsāCȉǐ̕aԂɏœ_ĂÖ@̊J̌̒SIȉۑɂȂƎvD

<h2>͂߂</h2>
@ǂƂaԂ̊TO͔ɍL͈͂łCǂ͌Â瑽̎Ɋ^ĂƍlĂDŁCǂ̂鎾͑ɂ킽ɂ炸C̕q@̏ڍׂ͂Ȃ炭炩ɂĂȂDƂ낪CƑ̉ǐɂ錴`q̔ЂƂ̌_@Ƃ<a href="#R1"><sup>1,2)</sup></a>CǂU镪q@\̏ڍׂЂƂ<a href="#R3"><sup>3)</sup></a>D܂ɁCᇂ_oϐȂǂ̂悤ɁC܂ŉǂƂ̊֘Amł͂ȂƉǂƂ̊֘AɂĂ񍐂悤ɂȂĂ<a href="#R4"><sup>4)</sup></a>Dȏ̂悤ȔwiCǂ͂܂ōlĂȏɕL̊ՂƂȂCaԂ̐iWɊ֗^Ăƍl悤ɂȂĂD̍lɂƂÂC̈ÂɂẮCǂ̂悤ɉǂ𐧌䂷邩ƂƂÂl邤ŒSIȉۑɂȂ낤ƂĂD
@ǂُ͓̈CCŕȂǊO̕ȂǂɂUƍlChɈˑĔזEVOi`BoHЂN鉊ǂ̕aԂlłDƉunُ̈ɂU鎾ƂČÂ玩ȖƉůTOmĂCȖƉu͎ȍRFTזEBזEߏɊ邢͑B邱Ƃɂ蔭ǂƍlĂDCŋ߂̌CȖƉu▾炩Ȋ݂Ȃɂ炸CRƉun𐧌䂷זẺߏȊ_@Ƃ錃ǂƂ鎾oC͎ȉǐƒ`ꂻ̊TOm<a href="#R1"><sup>1,5)</sup></a>Dȉǐ́C`ɂ͒P̈`qُ̈ɂ蔭ǂ`̎ƂC`炩ł͂ȂC邢́CqɔǂƍlĂ鎾̂ȂɂȉǐƂĕނ̉\ȎƎvDɁCȉǐUVOi`BoH͂̐iW̉ߒɊ^Ăƍl邱ƂCȉǂ͑ɂ킽鎾Ɋ^ĂƂi<a href="#F1">}1</a>jD

<a name="F1"></a><div id="fig1-caption-text" style="display: none;"><strong>}1@ȉǐ̊TO</strong>
ȉǐ́C`ɂ͒P`̎ƍlĂ邪Cl̕aԂ́Cq̎ɂ^Ă邱Ƃ炩ɂȂĂD
<a href="http://leading.lifesciencedb.jp/wordpress/wp-content/uploads/2014/11/Yasutomo-3.e013-Fig.1.jpg" target="_blank">[Download]</a></div>[hs_figure id=1&amp;image=/wordpress/wp-content/uploads/2014/11/Yasutomo-3.e013-Fig.1.png&amp;caption=fig1-caption-text]

@ȉǐ̌̐iẂCNLRP3Ȃǂ̒P̈qُ̈ɋN鎾̕qaԂ̔_@ƂȂ蔭WĂ<a href="#R1"><sup>1,2,6-11)</sup></a>D̂悤ȒP̈`qψقɂ鎾Ɋւ錤hāCŋ߁CNLRP3͓Aa邢̓AcnC}[a̕aԂɂ^邱Ƃ炩ɂȂD̃r[ł́Cȉǐ̔ǂɊ^镪q̏ڍׂƂ̕aԂɂĉD

<h2>1D<em>NLRP3</em>`q̕ψقɋN鎩ȉǐ</h2>
@Ct}\[͍זEɐNa邢͔A_Ȃǂ̌ɂ芈^pN̂̑̂łDʓIɂ́CCt}\[̊ɂJXp[[1ÔJXp[[1ɕϊCC^[CL1C^[CL18Ȃǂ̉ǐTCgJC̎YU邱Ƃɂ艊ǂN邱ƂmĂ<a href="#R5"><sup>5)</sup></a>DCt}\[\^pNƂĂNODlêmĂDNODlẽt@~[͍זEɊF^pNƂĐỉߒɂđllĂƐDł́CNODlẽt@~[̂CȉǐƂ̊֘A[NLRP3ɂĉD
@NLRP3Ct}\[̓A~ChCA_̌CזEOATPɂ芈邱ƂmĂ<a href="#R12"><sup>12,13)</sup></a>DC̕qǂ̂悤ȕq@ɂNLRP3Ct}\[邩ɂĂ̏ڍׂ͂܂ĂȂDCK<sup>+</sup>̗o⃊\\[̑Ȃǂ̋@lĂCɍŋ߂ł́C~gRhÅ֗^Ȃǂ̏dvȒm񍐂ĂDK<sup>+</sup>̊֗^ɊւẮCK<sup>+</sup>זEOɗo邱ƂɂזEK<sup>+</sup>ZxቺNLRP3Ct}\[邱ƂĂ<a href="#R14"><sup>14)</sup></a>DۂɁCK<sup>+</sup>̗o}NLRP3Ct}\[̊}邱Ƃ炩ɂĂDZxATPNLRP3Ct}\[邪CATPɂP2X7êK<sup>+</sup>̗oЂN邱ƂɂƍlĂ<a href="#R15"><sup>15)</sup></a>D\\[NLRP3Ct}\[Ƃ̊֌WɊւẮC\\[̑NLRP3Ct}\[̊Ɋ֗^邱Ƃ񍐂Ă<a href="#R13"><sup>13,16)</sup></a>D\\[̔jɂ胊\\[̓ɋǍ݂^pNזEɕo邱ƂɂNLRP3Ct}\[͊ƍlĂDCK<sup>+</sup>̗oу\\[̑ǂ̂悤ȕq@\ɂNLRP3Ct}\[邩ɂĂ̏ڍׂ͖炩ł͂ȂD~gRhÅ֗^ɂẮCNLRP3Ct}\[邽߂̑`邱ƁC~gRhAɗR镪qNLRP3Ct}\[̊Ɋ֗^邱ƂmĂD2012NC~gRhAɂYꂽ_f~gRhADNA_CזEɈڍsNLRP3ƌ邱Ƃ񍐂ꂽ<a href="#R17"><sup>17)</sup></a>D~gRhA𑫏ɂƂ_ł́CMVASNLRP3Ɖ邱ƂNLRP3Ct}\[̊ɂďdvł邱Ƃ񍐂ꂽ<a href="#R18"><sup>18)</sup></a>DCMVASזEłATPɑ΂锽F߂ȂCVJȂǂ̌ɑ΂锽͐ɗU邱ƂCNLRP3Ct}\[̊̃VOi`BoHƂĕ݂ĂChɂgĂ邱ƂD܂CNLRP3Ct}\[̊ɂ͔ǂ䂪Kvł邱Ƃ񍐂<a href="#R19"><sup>19)</sup></a>CNLRP3Ct}\[̊ɂ͓Iȑ̌`Kvł邱Ƃ炩ɂȂD
@<em>NLRP3</em>`q̕ψقɋN鎾͈`^ɂ肻̏Ǐ󂪈قȂCƑ`ȉǐǌQCMuckle-WellsǌQCVǑ퐫ǐ3ނɑʂDF̗D̈``ŔǂƑ`ȉǐǌQMuckle-WellsǌQɂāC<em>NLRP3</em>`q̊^̕ψق肳ꂽ<a href="#R20"><sup>20)</sup></a>DƑ`ȉǐǌQ͊hɂC݂ƂȂȂ܂l̔]C֐ߒɁCMƂ鎾łDhǂ̂悤ȕq@\ɂNLRP3Ct}\[邩ɂĂ̏ڍׂ͗ĂȂDMuckle-WellsǌQ͔]C֐ߒɁCMɂ킦C̓tA~Ch[VXƂC̔ǂɂ͕KhKvƂȂD<em>NLRP3</em>`q̃weȕψقɂ蔭ǂ邱Ƃ炩ɂȂĂC킪ł̉ƌn񍐂Ă<a href="#R21"><sup>21)</sup></a>D܂C<em>NLRP3</em>`q̕ψق̃p^[ɈˑāCyǌ^łƑ`ȉǐǌQdǌ^łMuckle-WellsǌQւ̈ȀǏ󂪂ЂNƍlDɁCVǑ퐫ǐ͂dǂ̎łD̕aԂ́C<em>NLRP3</em>`q̊^̕ψقɂJXp[[1C^[CL1ߏɎY邱ƂɈˑƍlĂCCRC^[CL1Ö@ɂ茰̌ʂ邱Ƃ񍐂ĂD
@<em>NLRP3</em>`qɐBזEψق̔F߂ȂVǑ퐫ǐ̖70<em>NLRP3</em>`q̕ψق̍זEUCNŔǂƂڂׂm񍐂ꂽ<a href="#R22"><sup>22)</sup></a>D܂CMuckle-WellsǌQɂĂl̑̍זEUCN񍐂ꂽ<a href="#R23"><sup>23)</sup></a>D<em>NLRP3</em>`q̕ψقזE̐Ŝ̍זEɑ΂ႢłĂCߏȃC^[CL1Y邱ƂaԂ̖{Ԃł邽ߔǂ邱ƂlD
@ɕ͌t̔A_lɂȂC֐߂̎⌃ɂ݂ƂȂłDɕƂĒmˑŘ֐߂̒ɂ݂̓́C֐ߍoւ̍D̗łDȑOCA_igĚ̂ЂNǂɕ̕aԂ̊Ղł邱Ƃ͒mĂČƂȂVOi`BoH͕słD2006NCNLRP3VOi`BoHA_igEɂ芈ČʂƂĒɕǂ邱Ƃ񍐂ꂽ<a href="#R12"><sup>12)</sup></a>DCNLRP3mbNAEg}EXł͔A_igEɂC^[CL1̎Y̘i͊ώ@ȂƂCA_igENLRP3Ƃ̊֘AꂽDCA_igEǂ̂悤ȕq@\ɂNLRP3Ct}\[邩ɂĂ͂܂ɕsȓ_CA_igE̔F̋@\тɂЂÂNLRP3Ct}\[̊̋@\̉𖾂͍̉ۑƂD
@AcnC}[a͎vl\͂LȂǂQisst̔]łD60Έȍ~ɔǂ邱ƂCFmǂ̌̂ЂƂłDAcnC}[å҂̔]ɂ̓A~ChƂ΂鑽ُ̈ȋÏŴĂ邱ƂmĂDAcnC}[å҂̔]gDɂăJXp[[1̊ώ@ꂽ<a href="#R4"><sup>4)</sup></a>DɁCƑAcnC}[aɂ݂`qψقJXp[[1mbNAEg}EX邢NLRP3mbNAEg}EX́CԋLȂǂ̏QサĂD܂CNLRP3̌ɂ~NOA̕\^ωCA~Ch̒}邱Ƃ炩ɂȂDȏ̌ʂCNLRP3Ct}\[̓AcnC}[a̐isɊ֗^Ă邱ƂꂽDNLRP3VOi`BoH̗}̓AcnC}[a̎ÂɗLł\Cis̔]̑\łAcnC}[a̎Ö@̊JɊ҂D
@bHȂǂɂ얞CXRU邱ƂmĂ邪Cꂪǂ̂悤ȕq@ɂ͕̂słDŋ߂̌ɂāCNLRP3CXRɊ֗^邱Ƃ񍐂ꂽDXA~Ch|yv`hƂ2^Aa̔ǂɕtXɒ邱ƂmĂA~Ch̃IS}[CNLRP3VOi`BoHăC^[CL1̎Y𘴐i邱Ƃoꂽ<a href="#R24"><sup>24)</sup></a>D܂C2^Aa̎ÖłOuhXA~Ch|yv`hɂC^[CL1̎Y}邱Ƃ炩ɂꂽD܂CɂƂLxɑ݂Oab_łp~`_NLRP3Ct}\[邱ƂɂCXRЂN邱Ƃoꂽ<a href="#R25"><sup>25)</sup></a>Dp~`_̓|ɂhꂽ}Nt@[W̃C^[CL1̎Y𘴐i邪CNLRP3邢̓JXp[[1}Nt@[Wł͂̂悤Și͊ώ@ȂDɁCNLRP3mbNAEg}EXɍbHĂϓ\уCX󐫂͈ȂƂꂽDp~`_ǂ̂悤ȕq@\ɂNLRP3Ct}\[̂ɂẮCp~`_ƃ|ɂAMPLi[[̃_ቺCɂI[gt@W[ቺ邱ƂoꂽDɁCp~`_̓~gRhÅ_f̎Y𘴐iĂD܂Cp~`_у|AMPLi[[̑jQăI[gt@W[}C~gRhÅ_f̎Y𘴐i邱ƂɂNLRP3Ct}\[ĂƍlꂽD

<h2>2DƉuveA\[̋@\̕sSɋN鎩ȉǐ</h2>
@60Nȏ܂C킪ɂāCis̎b̈ޏkэMƂ鎾񍐂C-ǌQƂ΂ꂽD̎͌Â炻݂̑mĂ̂́Č͕słDM҂́CVяHcɓl݂̎邱ƂmC̊҂͎ȍR̂ȂƁCՊȂƁCǂ邱ƁCƂ_ŎȉǐɕނꂤƍlD҂̓IȗՏǏ󂨂ьƂāCMCߐgCOuǁCC^pN̍lɂ킦CȐis̎b̈ޏk؂̈ޏkȂǂꂽDM҂́C̎Japanese autoinflammatory syndrome with lipodystrophyiJASLjƖ<a href="#R9"><sup>9)</sup></a>Cގ̏Ǐ悷鎾ƂāC-ǌQ̂قCCOɂĂJMPǌQCANDLEǌQmĂ<a href="#R8"><sup>8-11)</sup></a>D
@{l2̉ƌnpA͂уzڍ̃}bsOɂCJASĽ`q͑6F̂ɑ݂ƍlꂽD̗̈ɏœ_ĂGN\[͂̌ʁCƂȂ`qψق<em>PSMB8</em>`q̃GL\5ɓ肵<a href="#R9"><sup>9)</sup></a>D̕ψق̓A~m_uƂȂ~XZXψقłD̃A~m_c͎ĕۑĂƂC@\э\̈ێɏdvłƐꂽD<em>PSMB8</em>`q̓C^[tFɂ蔭̗UveA\[̍\^pNł5iR[h`qƂĕ񍐂Ă<a href="#R26"><sup>26)</sup></a>D-ǌQCJMPǌQCCANDLEǌQ̂<em>PSMB8</em>`q̕ψقɂ蔭ǂ邱Ƃ񍐂<a href="#R8"><sup>8-11)</sup></a>C킪ɂψق͂̂łCCOŕ񍐂ꂽψق͈قȂ镔ʂ̕ψقłDCl̏Ǐ悷邪<em>PSMB8</em>`qɕψق݂̑ȂǗ݂̑邱Ƃ炩ɂĂD
@veA\[̓rL`^pN𕪉^pNyf̂łDveA\[̓OɃ^pNyf̊TujbgƂ{\C݂܂łɁC\IɔWveA\[̂قCƉuveA\[ыBveA\[mĂ<a href="#R27"><sup>27,28)</sup></a>DWveA\[͂قƂǂׂĂ̍זEɔC\^pNł1C2C5^pNyfDWveA\[̃1C2C51iC2iC5iɒûƉuveA\[łDWveA\[ƓlɁC1iC2iC5i^pNyfĈ5i͋LgvVl̊ƂmĂDɏqׂ悤ɁC<em>PSMB8</em>`q͂̃5iR[hDƉuveA\[MHCNXIɒ񎦂yv`h悭؂oƂ񍐂Ă<a href="#R27"><sup>27)</sup></a>DŁC5imbNAEg}EXCсC1iC2iC5ĩgvmbNAEg}EXɂĉǂ͊ώ@ĂȂ<a href="#R29"><sup>29)</sup></a>D̒ḿCqgƃ}EXɂĖƉuveA\[̐䂷@\قȂ邱ƁC邢́CȊO̖m̋@\̂邱ƂD̏ꍇCƉuveA\[̈`qψقɋN鎩ȉǐ݂̑́CP퐫̈ێɂƉuveA\[̋@\IȖ𖾂炩ɂ邤ł߂ċMdȒmłƍliveA\[ɂĂ̏ڍׂ́C<a href="http://leading.lifesciencedb.jp/3-e011/" target="_blank">c Ε, ̈Zr[, <strong>3</strong>, e011, 2014</a> QƂꂽjD
@JASLɂĔꂽ<em>PSMB8</em>`q̕ψق̓~XZXψقłC̕ψقƉuveA\[̋@\ُ̈ƂɂāC҂sBזEɂă5i̔ɒቺĂ邱Ƃ炩ɂȂD܂CJASLɗRsBזEɂẮC5iƂĐ䂷LgvVl̊ł͂ȂCgvVl̊уJXp[[l̊̂ቺĂCׂẴ^pNyf̒ቺĂ邱Ƃ炩ɂȂDł́Cǂ̂悤ȕq@\ɂ肷ׂẴ^pNyfቺ̂ł낤DveA\[ɂĂ͂̂̂̃Tujbgɂ镡̂̌`Ɍɐ䂳ĂDŁCψٌ^̃5iɂveA\[̕qWω邩ǂɂČĎʁCψٌ^̃5ȋ݂ɂƉuveA\[̕qW͑jQC5i܂܂ȂqWԑ̂邱Ƃ炩ɂȂDȏ̌ʂC<em>PSMB8</em>`q̃~XZXψقɂƉuveA\[̕qWjQ邱ƂꂽDۂɁCJASL̊҂̔畆gDł͍זEъjɂărL`^pN̒~ςώ@ꂽD
@PSMB8mbNAEg}EXɂ͉ǂ̎Rǂ͕񍐂ĂȂƂCǂǂ̂悤ȋ@ɂUĂ邩Ƃ_dvȉۑłDJASL̊҂̔畆ɂĂ̓C^[CL6̔㏸ĂCJASL̊҂ɗRsBזEPMAуCIm}CVɂhƁCΏƂƂȂזEɔ׃C^[CL6̔Lӂɏ㏸D܂C̃C^[CL6̔̏㏸p38jQ܂ɂ}ꂽDȏ̂ƂCƉuveA\[̋@\ቺ邱ƂɂCߏp38ăC^[CL6̔Ăƍlꂽi<a href="#F2">}2</a>jD܂ł̂ƂCƉuveA\[̋@\ُ̈킪ǂ̂悤ȕq@\ɂp38邩ɂĂ͖炩ɂĂȂDCC^[tFVOi`BoHĂƂ񍐂邱ƂC̃VOi`BoH͂炭Ƃɂ艊ǂUĂƍlDƉuveA\[̋@\̒ቺɂCǂ̂悤ȕq@ɂẽTCgJC̎YǂU邩ɂẮCJASL̓f邱ƂȂǂɂ薾炩ɂKvƎvD

<a name="F2"></a><div id="fig2-caption-text" style="display: none;"><strong>}2@ƉuveA\[̋@\ُ̈Ɖ</strong>
iajȖƉuveA\[D
ibjψٌ^̃5iזEɂƉuveA\[̋@\ُ̈Dψٌ^̃5iɂƉuveA\[̕qWjQC̊ቺĎʁCזEɃrL`^pN~ςDψٌ^̃5iזEłp38C^[CL6̎Yi邪CrL`^pN̒~ςǂ̂悤ȕq@\ɂp38邩ɂĂ͉𖾂ĂȂD
<a href="http://leading.lifesciencedb.jp/wordpress/wp-content/uploads/2014/11/Yasutomo-3.e013-Fig.2.jpg" target="_blank">[Download]</a></div>[hs_figure id=2&amp;image=/wordpress/wp-content/uploads/2014/11/Yasutomo-3.e013-Fig.2.png&amp;caption=fig2-caption-text]

@JASL̓IȏǏ̂ЂƂ͐is̎b̈ޏkłDM҂́C5i̋@\̒ቺbזE̕}邱ƂCb̈ޏǩ̂ЂƂł邱Ƃ񍐂<a href="#R9"><sup>9)</sup></a>DJASLɂ鎉b̈ޏk͖Ɖu}܂Ȃǂɂ莩ȉǂPĂis邱ƂCՏIɂ͔ɏdvȖɂȂDbזËُ̕킾b̈ޏkɊ֗^Ă̂C邢́CǂՏIɉPĂb̈ޏk͐is邱Ƃ牊ǂ̊֗^͒ႢƂl邪Cǂǂ̂炢b̈ޏkɊ֗^Ă邩ɂĖ炩ɂKvD

<h2></h2>
@ȉǐƂĕނ鎾̂قɂCȉǂ̐iWɂ鎾͑lłƍlD`̎ȉǐ̌Cȉǂɂ`qƂNODlẽt@~[ɂ킦CƉuveA\[̍\^pN炩ɂꂽD炭CȊOɂlȃVOi`BoHȉǐɊ^ĂƍlCCɈ`wIȉ͂ɂ茴ƂȂ`IȑfɊւ錤iނƍlD܂C̃VOi`BoHՒf悤ȎÖ@̊JC̉ǐ̎Ö@̊J̒SIȉۑ̂ЂƂɂȂƗ\D

<h2> </h2>
<ol>
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</ol>
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