@article{Maccari_Fuchs_Kury-disti_popul_and-2021,
author = {Maccari, Maria Elena and Fuchs, Sebastian and Kury, Patrick 
          and Andrieux, Geoffroy and Volkl, Simon and Bengsch, Bertram 
          and Lorenz, Myriam Ricarda and Heeg, Maximilian and Rohr, 
          Jan and Jagle, Sabine and Castro, Carla N. and Gross, Miriam 
          and Warthorst, Ursula and Konig, Christoph and Fuchs, Ilka 
          and Speckmann, Carsten and Thalhammer, Julian and Kapp, 
          Friedrich G. and Seidel, Markus G. and Duckers, Gregor and 
          Schonberger, Stefan and Schutz, Catharina and Fuhrer, Marita 
          and Kobbe, Robin and Holzinger, Dirk and Klemann, Christian 
          and Smisek, Petr and Owens, Stephen and Horneff, Gerd and 
          Kolb, Reinhard and Naumann-Bartsch, Nora and Miano, Maurizio 
          and Staniek, Julian and Rizzi, Marta and Kalina, Tomas and 
          Schneider, Pascal and Erxleben, Anika and Backofen, Rolf and 
          Ekici, Arif and Niemeyer, Charlotte M. and Warnatz, Klaus 
          and Grimbacher, Bodo and Eibel, Hermann and Mackensen, 
          Andreas and Frei, Andreas Philipp and Schwarz, Klaus and 
          Boerries, Melanie and Ehl, Stephan and Rensing-Ehl, Anne},
title = {A distinct {CD38}+{CD45RA}+ population of {CD4}+, {CD8}+, 
         and double-negative {T} cells is controlled by {FAS}},
journal = {J Exp Med},
year = {2021},
doi = {10.1084/jem.20192191},
volume = {218},
user = {backofen},
pmid = {33170215},
pages = {},
number = {2},
issn = {0022-1007},
abstract = {The identification and characterization of rare immune cell 
            populations in humans can be facilitated by their growth 
            advantage in the context of specific genetic diseases. Here, 
            we use autoimmune lymphoproliferative syndrome to identify a 
            population of FAS-controlled TCRalphabeta+ T cells. They 
            include CD4+, CD8+, and double-negative T cells and can be 
            defined by a CD38+CD45RA+T-BET- expression pattern. These 
            unconventional T cells are present in healthy individuals, 
            are generated before birth, are enriched in lymphoid tissue, 
            and do not expand during acute viral infection. They are 
            characterized by a unique molecular signature that is 
            unambiguously different from other known T cell 
            differentiation subsets and independent of CD4 or CD8 
            expression. Functionally, FAS-controlled T cells represent 
            highly proliferative, noncytotoxic T cells with an IL-10 
            cytokine bias. Mechanistically, regulation of this 
            physiological population is mediated by FAS and CTLA4 
            signaling, and its survival is enhanced by mTOR and STAT3 
            signals. Genetic alterations in these pathways result in 
            expansion of FAS-controlled T cells, which can cause 
            significant lymphoproliferative disease.}
}