FAS

Tumor necrosis factor receptor superfamily member 6 ;
Apo-1 antigen ;
Apoptosis-mediating surface antigen FAS ;
FASLG receptor ;
CD antigen CD95 ;

disease:Defects in FAS are the cause of autoimmune lymphoproliferative syndrome type 1A (ALPS1A) [MIM:601859]; also known as Canale-Smith syndrome (CSS). ALPS is a childhood syndrome involving hemolytic anemia and thrombocytopenia with massive lymphadenopathy and splenomegaly.,domain:Contains a death domain involved in the binding of FADD, and maybe to other cytosolic adapter proteins.,function:Receptor for TNFSF6/FASLG. The adapter molecule FADD recruits caspase-8 to the activated receptor. The resulting death-inducing signaling complex (DISC) performs caspase-8 proteolytic activation which initiates the subsequent cascade of caspases (aspartate-specific cysteine proteases) mediating apoptosis. FAS-mediated apoptosis may have a role in the induction of peripheral tolerance, in the antigen-stimulated suicide of mature T-cells, or both. The secreted isoforms 2 to 6 block apoptosis (in vitro).,online information:Mutations in TNFRSF6 causing ALPS type Ia,similarity:Contains 1 death domain.,similarity:Contains 3 TNFR-Cys repeats.,subunit:Binds DAXX. Interacts with HIPK3. Part of a complex containing HIPK3 and FADD (By similarity). Binds RIPK1 and FAIM2. Interacts with BRE and FEM1B.,tissue specificity:Isoform 1 and isoform 6 are expressed at equal levels in resting peripheral blood mononuclear cells. After activation there is an increase in isoform 1 and decrease in the levels of isoform 6.,

cell activation, leukocyte homeostasis, adaptive immune response, immune effector process, lymphocyte homeostasis,immunoglobulin production, production of molecular mediator of immune response, leukocyte mediated immunity,lymphocyte mediated immunity, adaptive immune response based on somatic recombination of immune receptors built from immunoglobulin superfamily domains, immune system development, leukocyte differentiation, negative regulation of immune system process, regulation of leukocyte activation, negative regulation of leukocyte activation, renal system process, protein complex assembly, apoptosis, anti-apoptosis, induction of apoptosis, activation-induced cell death of T cells, inflammatory cell apoptosis, transformed cell apoptosis, immune response, cell death, induction of apoptosis by extracellular signals, activation of pro-apoptotic gene products, response to toxin, response to endogenous stimulus, response to hormone stimulus, response to organic substance, regulation of necrotic cell death,positive regulation of necrotic cell death, regulation of cell death, positive regulation of cell death, programmed cell death, induction of programmed cell death, death, B cell mediated immunity, hemopoiesis, lymphocyte differentiation, T cell differentiation, response to corticosteroid stimulus, T cell differentiation in the thymus, T cell activation,homeostatic process, regulation of apoptosis, T cell homeostasis, positive regulation of apoptosis, negative regulation of apoptosis, regulation of programmed cell death, positive regulation of programmed cell death, negative regulation of programmed cell death, negative T cell selection, macromolecular complex subunit organization, T cell selection,negative thymic T cell selection, thymic T cell selection, leukocyte activation, regulation of lymphocyte differentiation,regulation of myeloid cell differentiation, lymphocyte activation, hemopoietic or lymphoid organ development, spleen development, response to steroid hormone stimulus, homeostasis of number of cells, regulation of B cell activation,regulation of cell activation, negative regulation of cell activation, negative regulation of B cell activation, regulation of lymphocyte activation, negative regulation of lymphocyte activation, protein oligomerization, protein homooligomerization, response to glucocorticoid stimulus, response to protein stimulus, negative regulation of cell death, macromolecular complex assembly, lymphocyte apoptosis, T cell apoptosis, protein complex biogenesis,

[Isoform 1]: Cell membrane ; Single-pass type I membrane protein . Membrane raft .; [Isoform 2]: Secreted.; [Isoform 3]: Secreted.; [Isoform 4]: Secreted.; [Isoform 5]: Secreted.; [Isoform 6]: Secreted.

Isoform 1 and isoform 6 are expressed at equal levels in resting peripheral blood mononuclear cells. After activation there is an increase in isoform 1 and decrease in the levels of isoform 6.