Article
ALPS: an autoimmune human lymphoproliferative syndrome associated with abnormal lymphocyte apoptosis.
Seminars in immunology - 1 Feb 1997
Puck J M, Sneller M C
Abstract excerpt
Apoptosis of activated lymphocytes is critical to immune homeostasis. The cell surface receptor Fas is an important mediator of lymphocyte apoptosis; defective Fas expression causes accumulation of lymphocytes and autoimmune disease in mice. Apoptosis defects due to mutations of Fas have also been found in a rare human autoimmune lymphoproliferative syndrome (ALPS). Nine unrelated children with ALPS had...
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