免疫原
Purified recombinant human FAS (C-terminus) protein fragments expressed in E.coli.
特异性
This antibody detects endogenous levels of FAS (C-terminus) and does not cross-react with related proteins.
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
纯化工艺
The antibody was affinity-purified from mouse ascites by affinity-chromatography using epitope-specific immunogen.
其他名称
ALPS 1A;ALPS1A;APO 1;Apo 1 antigen;APO 1 cell surface antigen;Apo-1 antigen;APO1;Apo1 antigen;APO1 cell surface antigen;Apoptosis antigen 1;Apoptosis mediating surface antigen FAS;Apoptosis-mediating surface antigen FAS;APT 1;APT1;CD 95;CD 95 antigen; CD95;CD95 antigen;Delta Fas;Delta Fas/APO 1/CD95;Delta Fas/APO1/CD95;Fas (TNF receptor superfamily, member 6);FAS 1;FAS 827dupA;Fas AMA;FAS;FAS Antigen;Fas cell surface death receptor;FAS1;FASLG receptor;FASTM;sFAS;Surface antigen APO1;TNF receptor superfamily, member 6;TNFRSF 6;TNFRSF6;TNR6_HUMAN;Tumor necrosis factor receptor superfamily member 6.
背景
The protein encoded by this gene is a member of the TNF-receptor superfamily. This receptor contains a death domain. It has been shown to play a central role in the physiological regulation of programmed cell death, and has been implicated in the pathogenesis of various malignancies and diseases of the immune system. The interaction of this receptor with its ligand allows the formation of a death-inducing signaling complex that includes Fas-associated death domain protein (FADD), caspase 8, and caspase 10. The autoproteolytic processing of the caspases in the complex triggers a downstream caspase cascade, and leads to apoptosis. This receptor has been also shown to activate NF-kappaB, MAPK3/ERK1, and MAPK8/JNK, and is found to be involved in transducing the proliferating signals in normal diploid fibroblast and T cells. Several alternatively spliced transcript variants have been described, s
功能
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 vit