英文名称
CD59 Recombinant Rabbit mAb
英文别名
CD59_HUMAN; CD59; 1F5 antigen; 20 kDa homologous restriction factor (HRF-20 | HRF20); MAC-inhibitory protein (MAC-IP); MEM43 antigen; Membrane attack complex inhibition factor (MACIF); Membrane inhibitor of reactive lysis (MIRL); Protectin; MIC11; MIN1; MIN2; MIN3; MSK21; CD59A_MOUSE; Cd59a; CD59 molecule (CD59 blood group); CD59 antigen p18-20 (antigen identified by monoclonal antibodies 16.3A5, EJ16, EJ30, EL32 and G344); CD59 antigen, complement regulatory protein; CD59 molecule, complement regulatory protein; CD59 molecule; CD59 glycoprotein; membrane attack complex inhibition factor; membrane inhibitor of reactive lysis
免疫原
KLH conjugated synthetic peptide derived from human CD59
纯化方法
affinity purified by Protein A
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
产品介绍
反应性溶血膜抑制蛋白（CD59）是血细胞膜上糖化磷脂酰肌醇（GPI）锚定蛋白，具有抑制补体系统激活，参与信号传递，有协助T淋巴细胞活化功能,CD59在补体调节过程中起着很主要的作用。
背景资料
This gene encodes a cell surface glycoprotein that regulates complement-mediated cell lysis, and it is involved in lymphocyte signal transduction. This protein is a potent inhibitor of the complement membrane attack complex, whereby it binds complement C8 and/or C9 during the assembly of this complex, thereby inhibiting the incorporation of multiple copies of C9 into the complex, which is necessary for osmolytic pore formation. This protein also plays a role in signal transduction pathways in the activation of T cells. Mutations in this gene cause CD59 deficiency, a disease resulting in hemolytic anemia and thrombosis, and which causes cerebral infarction. Multiple alternatively spliced transcript variants, which encode the same protein, have been identified for this gene. [provided by RefSeq, Jul 2008]
亚基
Interacts with T-cell surface antigen CD2.
亚细胞定位
Cell membrane; Lipid-anchor, GPI-anchor. Secreted. Note=Soluble form found in a number of tissues.
翻译后修饰
N- and O-glycosylated. The N-glycosylation mainly consists of a family of biantennary complex-type structures with and without lactosamine extensions and outer arm fucose residues. Also significant amounts of triantennary complexes (22%). Variable sialylation also present in the Asn-43 oligosaccharide. The predominant O-glycans are mono-sialylated forms of the disaccharide, Gal-beta-1,3GalNAc, and their sites of attachment are probably on Thr-76 and Thr-77. The GPI-anchor of soluble urinary CD59 has no inositol-associated phospholipid, but is composed of seven different GPI-anchor variants of one or more monosaccharide units. Major variants contain sialic acid, mannose and glucosamine Sialic acid linked to an N-acetylhexosamine-galactose arm is present in two variants.
Glycated. Glycation is found in diabetic subjects, but only at minimal levels in nondiabetic subjects. Glycated CD59 lacks MAC-inhibitory function and confers to vascular complications of diabetes.
疾病
CD59 deficiency (CD59D) [MIM:612300]: A deficiency resulting in chronic complement-mediated intravascular hemolysis, anemia, hemoglobinuria and thrombosis. Note=The disease is caused by mutations affecting the gene represented in this entry.
相似性
Contains 1 UPAR/Ly6 domain.
功能
Potent inhibitor of the complement membrane attack complex (MAC) action. Acts by binding to the C8 and/or C9 complements of the assembling MAC, thereby preventing incorporation of the multiple copies of C9 required for complete formation of the osmolytic pore. This inhibitor appears to be species-specific. Involved in signal transduction for T-cell activation complexed to a protein tyrosine kinase.
The soluble form from urine retains its specific complement binding activity, but exhibits greatly reduced ability to inhibit MAC assembly on cell membranes.
