XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome

@article{Rigaud2006XIAPDI,
  title={XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome},
  author={St{\'e}phanie Rigaud and M. -C. Fondaneche and Nathalie F. Lambert and Benoit Pasquier and V{\'e}ronique Mat{\'e}o and Pauline Soulas and Lionel Galicier and Françoise le Deist and Fr{\'e}d{\'e}ric Rieux-Laucat and Patrick Revy and Alain Fischer and Genevi{\'e}ve de Saint Basile and Sylvain Latour},
  journal={Nature},
  year={2006},
  volume={444},
  pages={110-114}
}
The homeostasis of the immune response requires tight regulation of the proliferation and apoptosis of activated lymphocytes. In humans, defects in immune homeostasis result in lymphoproliferation disorders including autoimmunity, haemophagocytic lymphohystiocytosis and lymphomas. The X-linked lymphoproliferative syndrome (XLP) is a rare, inherited immunodeficiency that is characterized by lymphohystiocytosis, hypogammaglobulinaemia and lymphomas, and that usually develops in response to… CONTINUE READING
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