Hyperactive mTOR pathway promotes lymphoproliferation and abnormal differentiation in autoimmune lymphoproliferative syndrome.

@article{Voelkl2016HyperactiveMP,
  title={Hyperactive mTOR pathway promotes lymphoproliferation and abnormal differentiation in autoimmune lymphoproliferative syndrome.},
  author={Simon Voelkl and Anne Rensing-Ehl and Andrea Allg{\"a}uer and Elisabeth Schreiner and Myriam Ricarda Lorenz and Jan C. Rohr and Christian Klemann and Ilka Fuchs and Volker H Schuster and Andr{\'e} O von Bueren and Nora Naumann-Bartsch and Eleonora Gambineri and Kathrin Siepermann and Robin Kobbe and Michaela Nathrath and Peter D Arkwright and Maurizio Miano and Klaus-Daniel Stachel and Markus Metzler and Klaus Schwarz and Anita N Kremer and Carsten Speckmann and Stephan Ehl and Andreas Mackensen},
  journal={Blood},
  year={2016},
  volume={128 2},
  pages={227-38}
}
Autoimmune lymphoproliferative syndrome (ALPS) is a human disorder characterized by defective Fas signaling, resulting in chronic benign lymphoproliferation and accumulation of TCRαβ(+) CD4(-) CD8(-) double-negative T (DNT) cells. Although their phenotype resembles that of terminally differentiated or exhausted T cells, lack of KLRG1, high eomesodermin, and marginal T-bet expression point instead to a long-lived memory state with potent proliferative capacity. Here we show that despite their… CONTINUE READING
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Increased spontaneous in vitro apoptosis in double negative BLOOD, 14 JULY 2016 x VOLUME 128, NUMBER 2 HYPERACTIVE mTOR SIGNALING IN ALPS

JP Haas, M Grunke, C Frank
For personal use only. on September • 2016
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