XIAP deficiency in humans causes an X-linked lymphoproliferative syndrome

  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},
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
Highly Influential
This paper has highly influenced 19 other papers. REVIEW HIGHLY INFLUENTIAL CITATIONS
228 Citations
22 References
Similar Papers


Publications citing this paper.
Showing 1-10 of 228 extracted citations


Publications referenced by this paper.
Showing 1-10 of 22 references

Similar Papers

Loading similar papers…