Failure of Interferon γ to Induce the Anti-Inflammatory Interleukin 18 Binding Protein in Familial Hemophagocytosis 英文参考文献.docVIP

下载本文档

5
0
约2.95万字
约 6页
2017-05-11 发布于上海
举报
版权申诉

Failure of Interferon γ to Induce the Anti-Inflammatory Interleukin 18 Binding Protein in Familial Hemophagocytosis 英文参考文献.doc

1、本文档共6页，可阅读全部内容。
2、原创力文档（book118）网站文档一经付费（服务费），不意味着购买了该文档的版权，仅供个人/单位学习、研究之用，不得用于商业用途，未经授权，严禁复制、发行、汇编、翻译或者网络传播等，侵权必究。
3、本站所有内容均由合作方或网友上传，本站不对文档的完整性、权威性及其观点立场正确性做任何保证或承诺！文档内容仅供研究参考，付费前请自行鉴别。如您付费，意味着您自己接受本站规则且自行承担风险，本站不退款、不进行额外附加服务；查看《如何避免下载的几个坑》。如果您已付费下载过本站文档，您可以点击这里二次下载。
4、如文档侵犯商业秘密、侵犯著作权、侵犯人身权等，请点击“版权申诉”（推荐），也可以打举报电话：400-050-0827(电话支持时间：9:00-18:30)。
5、该文档为VIP文档，如果想要下载，成为VIP会员后，下载免费。
6、成为VIP后，下载本文档将扣除1次下载权益。下载后，不支持退款、换文档。如有疑问请联系我们。
7、成为VIP后，您将拥有八大权益，权益包括：VIP文档下载权益、阅读免打扰、文档格式转换、高级专利检索、专属身份标志、高级客服、多端互通、版权登记。
8、VIP文档为合作方或网友上传，每下载1次，网站将根据用户上传文档的质量评分、类型等，对文档贡献者给予高额补贴、流量扶持。如果你也想贡献VIP文档。上传文档

Failure of Interferon γ to Induce the Anti-Inflammatory Interleukin 18 Binding Protein in Familial Hemophagocytosis 英文参考文献

FailureofInterferonctoInducetheAnti-Inflammatory Interleukin18BindingProteininFamilial Hemophagocytosis ClaudiaA.Nold-Petry1,3,ThomasLehrnbecher2,AndreaJarisch2,DirkSchwabe2,JosefM.Pfeilschifter1, HeikoMuhl1,MarcelF.Nold1,3* 1PharmazentrumFrankfurt,JohannWolfgangGoethe-UniversityHospital,Frankfurt,Germany,2DepartmentofPediatrics,JohannWolfgangGoethe-UniversityHospital, Frankfurt,Germany,3DivisionofInfectiousDiseases,UniversityofColoradoDenver,Aurora,Colorado,UnitedStatesofAmerica Abstract Background:Familialhemophagocytosis(FHL)isararediseaseassociatedwithdefectsinproteinsinvolvedinCD8+T-cell cytotoxicity. Hyperactivation of immune cells results in a perilous, Th1-driven cytokine storm. We set out to explore the regulation of cytokines in an FHL patient who was clinically stable on low-dose immunosuppressive therapy after bone marrowtransplantationoverasix-monthperiod.Duringthisperiod,chimerismanalysesshowedthatthefractionofhost cellswasbetween1and10%.Bothparentsofthepatientaswellashealthyvolunteerswerestudiedforcomparison. Methods/Principal Findings: Using ELISA, quantitative real-time PCR, and clinical laboratory methods, we investigated constitutive and inducible cytokines, polymorphisms, and clinical parameters in whole blood and whole blood cultures. Althoughroutinelaboratorytestswerewithinthenormalrange,thechemokinesIP-10andIL-8aswellasthecytokineIL- 27p28wereincreasedupto10-foldunderconstitutiveandstimulatedconditionscomparedtohealthycontrols.Moreover, highlevelsofIFNcandTNFawereproduceduponstimulation.Unexpectedly,IFNcinductionofIL-18bindingprotein(IL- 18BP)wasmarkedlyreduced(1.6-foldvs5-foldincontrols).Thepatient’smotherfeaturedintermediatelyincreasedcytokine levels,whereaslevelsinthefatherweresimilartothoseinthecontrols. Conclusions/Significance:SinceIL-18playsamajorroleinperpetuatinghemophagocytosis,thefailureofIFNctoinduceIL- 18BPmayconstituteafundamentalpathogeneticmechanism.Furthermore,increasedproductionofIL-8andIL-27appears tobeassociatedwiththisdi