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Pyrin Activates the ASC Pyroptosome in Response to Engagement by Autoinflammatory PSTPIP1 Mutants

DC Field Value Language
dc.contributor.author유제욱-
dc.date.accessioned2014-12-21T17:12:37Z-
dc.date.available2014-12-21T17:12:37Z-
dc.date.issued2007-
dc.identifier.issn1097-2765-
dc.identifier.urihttps://ir.ymlib.yonsei.ac.kr/handle/22282913/97153-
dc.description.abstractThe molecular mechanism by which mutations in the cytoskeleton-organizing protein PSTPIP1 cause the autoinflammatory PAPA syndrome is still elusive. Here, we demonstrate that PSTPIP1 requires the familial Mediterranean fever protein pyrin to assemble the ASC pyroptosome, a molecular platform that recruits and activates caspase-1. We provide evidence that pyrin is a cytosolic receptor for PSTPIP1. Pyrin exists as a homotrimer in an autoinhibited state due to intramolecular interactions between its pyrin domain (PYD) and B-box. Ligation by PSTPIP1, which is also a homotrimer, activates pyrin by unmasking its PYD, thereby allowing it to interact with ASC and facilitate ASC oligomerization into an active ASC pyroptosome. Because of their high binding affinity to pyrin's B-box, PAPA-associated PSTPIP1 mutants were found to be more effective than WT PSTPIP1 in inducing pyrin activation. Therefore, constitutive ligation and activation of pyrin by mutant PSTPIP1 proteins explain the autoinflammatory phenotype seen in PAPA syndrome.-
dc.description.statementOfResponsibilityopen-
dc.format.extent214~227-
dc.relation.isPartOfMOLECULAR CELL-
dc.rightsCC BY-NC-ND 2.0 KR-
dc.rights.urihttps://creativecommons.org/licenses/by-nc-nd/2.0/kr/-
dc.titlePyrin Activates the ASC Pyroptosome in Response to Engagement by Autoinflammatory PSTPIP1 Mutants-
dc.typeArticle-
dc.contributor.collegeCollege of Medicine (의과대학)-
dc.contributor.departmentDept. of Microbiology (미생물학)-
dc.contributor.googleauthorJe-Wook Yu-
dc.contributor.googleauthorTeresa Fernandes-Alnemri-
dc.contributor.googleauthorEmad S. Alnemri-
dc.contributor.googleauthorZhiJia Zhang-
dc.contributor.googleauthorMargaret McCormick-
dc.contributor.googleauthorLeobaldo Solorzano-
dc.contributor.googleauthorChristine Juliana-
dc.contributor.googleauthorJianghong Wu-
dc.contributor.googleauthorPinaki Datta-
dc.identifier.doi10.1016/j.molcel.2007.08.029-
dc.admin.authorfalse-
dc.admin.mappingfalse-
dc.contributor.localIdA02508-
dc.relation.journalcodeJ02256-
dc.identifier.eissn1097-4164-
dc.identifier.urlhttp://www.sciencedirect.com/science/article/pii/S1097276507006429-
dc.contributor.alternativeNameYu, Je Wook-
dc.contributor.affiliatedAuthorYu, Je Wook-
dc.rights.accessRightsnot free-
dc.citation.volume28-
dc.citation.number2-
dc.citation.startPage214-
dc.citation.endPage227-
dc.identifier.bibliographicCitationMOLECULAR CELL, Vol.28(2) : 214-227, 2007-
dc.identifier.rimsid55210-
dc.type.rimsART-
Appears in Collections:
1. College of Medicine (의과대학) > Dept. of Microbiology (미생물학교실) > 1. Journal Papers

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