Dominant interfering fas gene mutations impair apoptosis in a human autoimmune lymphoproliferative syndrome

@article{Fisher1995DominantIF,
  title={Dominant interfering fas gene mutations impair apoptosis in a human autoimmune lymphoproliferative syndrome},
  author={G. H. Fisher and F. J. Rosenberg and S. Straus and J. Dale and L. Middelton and A. Lin and W. Strober and M. Lenardo and J. Puck},
  journal={Cell},
  year={1995},
  volume={81},
  pages={935-946}
}
  • G. H. Fisher, F. J. Rosenberg, +6 authors J. Puck
  • Published 1995
  • Biology, Medicine
  • Cell
  • Five unrelated children are described with a rare autoimmune lymphoproliferative syndrome (ALPS) characterized by massive nonmalignant lymphadenopathy, autoimmune phenomena, and expanded populations of TCR-CD3+CD4-CD8- lymphocytes. These findings, suggesting a genetic defect in the ability of T lymphocytes to respond to normal immunoregulatory mechanisms, prompted an evaluation of lymphocyte apoptosis. Each child had defective Fas-mediated T lymphocyte apoptosis associated with a unique… CONTINUE READING
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