Kühnle, Ingrid

[["dc.bibliographiccitation.firstpage","851"],["dc.bibliographiccitation.issue","6"],["dc.bibliographiccitation.journal","Blood"],["dc.bibliographiccitation.lastpage","860"],["dc.bibliographiccitation.volume","124"],["dc.contributor.author","Rensing-Ehl, Anne"],["dc.contributor.author","Voelkl, Simon"],["dc.contributor.author","Speckmann, Carsten"],["dc.contributor.author","Lorenz, Myriam Ricarda"],["dc.contributor.author","Ritter, Julia"],["dc.contributor.author","Janda, Ales"],["dc.contributor.author","Abinun, Mario"],["dc.contributor.author","Pircher, Hanspeter"],["dc.contributor.author","Bengsch, Bertram"],["dc.contributor.author","Thimme, Robert"],["dc.contributor.author","Fuchs, Ilka"],["dc.contributor.author","Ammann, Sandra"],["dc.contributor.author","Allgaeuer, Andrea"],["dc.contributor.author","Kentouche, Karim"],["dc.contributor.author","Cant, Andrew"],["dc.contributor.author","Hambleton, Sophie"],["dc.contributor.author","da Cunha, Claudia Bettoni"],["dc.contributor.author","Huetker, Sebastian"],["dc.contributor.author","Kuehnle, Ingrid"],["dc.contributor.author","Pekrun, Arnulf"],["dc.contributor.author","Seidel, Markus G."],["dc.contributor.author","Hummel, Michael"],["dc.contributor.author","Mackensen, Andreas"],["dc.contributor.author","Schwarz, Klaus"],["dc.contributor.author","Ehl, Stephan"],["dc.date.accessioned","2018-11-07T09:36:42Z"],["dc.date.available","2018-11-07T09:36:42Z"],["dc.date.issued","2014"],["dc.description.abstract","Accumulation of CD3(+) T-cell receptor (TCR)alpha beta(+)CD4(-)CD8(-) double-negative T cells(DNT) is a hallmark of autoimmune lymphoproliferative syndrome (ALPS). DNT origin and differentiation pathways remain controversial. Here we show that human ALPS DNT have features of terminally differentiated effector memory T cells reexpressing CD45RA(+) (TEMRA), but are CD27(+)CD28(+)KLRG1(-) and do not express the transcription factor T-bet. This unique phenotype was also detected among CD4(+) or CD8(+) ALPS TEMRA cells. T-cell receptor beta deep sequencing revealed a significant fraction of shared CDR3 sequences between ALPS DNT and both CD4(+) and CD8(+) TEMRA cells. Moreover, in ALPS patients with a germ line FAS mutation and somatic loss of heterozygosity, in whom biallelic mutant cells can be tracked by absent Fas expression, Fas-negative T cells accumulated not only among DNT, but also among CD4(+) and CD8(+)TEMRA cells. These data indicate that in human Fas deficiency DNT cannot only derive from CD8(+), but also from CD4(+) T cells. Furthermore, defective Fas signaling leads to aberrant transcriptional programs and differentiation of subsets of CD4(+) and CD8(+) T cells. Accumulation of these cells before their double-negative state appears to be an important early event in the pathogenesis of lymphoproliferation in ALPS patients."],["dc.identifier.doi","10.1182/blood-2014-03-564286"],["dc.identifier.isi","000342620400012"],["dc.identifier.pmid","24894771"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/32677"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Amer Soc Hematology"],["dc.relation.issn","1528-0020"],["dc.relation.issn","0006-4971"],["dc.title","Abnormally differentiated CD4(+) or CD8(+) T cells with phenotypic and genetic features of double negative T cells in human Fas deficiency"],["dc.type","journal_article"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.journal","Journal of Clinical Immunology"],["dc.bibliographiccitation.volume","34"],["dc.contributor.author","Rensing-Ehl, Anne"],["dc.contributor.author","Voelkl, Simon"],["dc.contributor.author","Speckmann, Carsten"],["dc.contributor.author","Lorenz, Myriam Ricarda"],["dc.contributor.author","Ritter, J."],["dc.contributor.author","Janda, Ales"],["dc.contributor.author","Abinun, Mario"],["dc.contributor.author","Pircher, Hanspeter"],["dc.contributor.author","Bengsch, B."],["dc.contributor.author","Thimme, Robert"],["dc.contributor.author","Fuchs, Ilka"],["dc.contributor.author","Ammann, Sandra"],["dc.contributor.author","Allgaeuer, Andrea"],["dc.contributor.author","Kentouche, Karim"],["dc.contributor.author","Cant, A."],["dc.contributor.author","Hambleton, Sophie"],["dc.contributor.author","da Cunha, Claudia Bettoni"],["dc.contributor.author","Huetker, Sebastian"],["dc.contributor.author","Kuehnle, Ingrid"],["dc.contributor.author","Pekrun, Arnulf"],["dc.contributor.author","Seidel, Markus G."],["dc.contributor.author","Hummel, Michael"],["dc.contributor.author","Mackensen, Andreas"],["dc.contributor.author","Schwarz, K."],["dc.contributor.author","Ehl, Stephan"],["dc.date.accessioned","2018-11-07T09:33:54Z"],["dc.date.available","2018-11-07T09:33:54Z"],["dc.date.issued","2014"],["dc.format.extent","S165"],["dc.identifier.isi","000347389100046"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/32064"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Springer"],["dc.publisher.place","New york"],["dc.relation.conference","16th Biennial Meeting of the European-Society-for-Immunodeficiencies"],["dc.relation.eventlocation","Prague, CZECH REPUBLIC"],["dc.relation.issn","1573-2592"],["dc.relation.issn","0271-9142"],["dc.title","Abnormally Differentiated CD4+OR CD8+T-Cells with Phenotypic and Genetic Features of Double Negative T-Cells in Human FAS Deficiency"],["dc.type","conference_abstract"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]

[["dc.bibliographiccitation.firstpage","73"],["dc.bibliographiccitation.journal","Journal of Clinical Immunology"],["dc.bibliographiccitation.lastpage","74"],["dc.bibliographiccitation.volume","32"],["dc.contributor.author","Rensing-Ehl, Anne"],["dc.contributor.author","Janda, Ales"],["dc.contributor.author","Vach, Werner"],["dc.contributor.author","Gladstone, Beryl P."],["dc.contributor.author","Abinun, Mario"],["dc.contributor.author","Albert, M. H."],["dc.contributor.author","Butler, Karina"],["dc.contributor.author","Cant, A."],["dc.contributor.author","Cseh, Anna-Maria"],["dc.contributor.author","Ebinger, Martin"],["dc.contributor.author","Goldacker, Sigune"],["dc.contributor.author","Hambleton, Sophie"],["dc.contributor.author","Hebart, Holger F."],["dc.contributor.author","Houet, Leonora"],["dc.contributor.author","Kentouche, Karim"],["dc.contributor.author","Kuehnle, Ingrid"],["dc.contributor.author","Lehmberg, Kai"],["dc.contributor.author","Mejstrikova, Ester"],["dc.contributor.author","Niemeyer, C."],["dc.contributor.author","Minkov, Milen"],["dc.contributor.author","Neth, O."],["dc.contributor.author","Dueckers, Gregor"],["dc.contributor.author","Owens, S."],["dc.contributor.author","Richards, M."],["dc.contributor.author","Roesler, J."],["dc.contributor.author","Schilling, Freimut H."],["dc.contributor.author","Schuster, Volker"],["dc.contributor.author","Seidel, Markus G."],["dc.contributor.author","Smisek, Petr"],["dc.contributor.author","Sukova, Martina"],["dc.contributor.author","Svec, Peter"],["dc.contributor.author","Wiesel, Thomas"],["dc.contributor.author","Lorenz, M."],["dc.contributor.author","Schwarz, K."],["dc.contributor.author","Ehl, Stephan"],["dc.contributor.author","Speckmann, Carsten"],["dc.date.accessioned","2018-11-07T09:06:30Z"],["dc.date.available","2018-11-07T09:06:30Z"],["dc.date.issued","2012"],["dc.identifier.isi","000308728900140"],["dc.identifier.uri","https://resolver.sub.uni-goettingen.de/purl?gro-2/25578"],["dc.notes.status","zu prüfen"],["dc.notes.submitter","Najko"],["dc.publisher","Springer"],["dc.publisher.place","New york"],["dc.relation.conference","15th Biennial Meeting European-Society-for-Immunodeficiency (ESID)"],["dc.relation.eventlocation","Florence, ITALY"],["dc.relation.issn","0271-9142"],["dc.title","WHEN SHOULD THE FAS GENE BE SEQUENCED IN PATIENTS WITH LYMPHOPROLIFERATION AND AUTOIMMUNITY"],["dc.type","conference_abstract"],["dc.type.internalPublication","yes"],["dc.type.peerReviewed","yes"],["dc.type.status","published"],["dspace.entity.type","Publication"]]