Clinical , Immunologic , and Genetic Features of an Autoimmune Lymphoproliferative Syndrome Associated With Abnormal Lymphocyte Apoptosis

@inproceedings{Sneller1997ClinicalI,
  title={Clinical , Immunologic , and Genetic Features of an Autoimmune Lymphoproliferative Syndrome Associated With Abnormal Lymphocyte Apoptosis},
  author={Michael C Sneller and Jin Wang and Janet K. Dale and Warren Strober and Lindsay A. Middelton and Youngnim Choi and Thomas A. Fleisher and M. S. Lim and Elaine S. Jaffe and Jennifer M Puck and Michael J Lenardo and Stephen E. Straus},
  year={1997}
}
Programmed cell death (apoptosis) of activated lymphoan unusual population of CD4CD8 T cells that express the a/b T-cell receptor (TCR). All patients showed defective lymcytes is critical to immune homeostasis. The cell surface protein Fas (CD95) and its ligand play a pivotal role in reguphocyte apoptosis in vitro. Heterozygous mutations of the Fas gene were detected in 8 patients. One ALPS patient lating lymphocyte apoptosis, and defective expression of either Fas or Fas ligand results in… CONTINUE READING