红细胞磷脂酰肌醇聚糖A抗体
规格：1mg/1ml
英文名： PIGA
别名： GlcNAc PI synthesis protein; GlcNAc-PI synthesis protein; GPI3; Phosphatidylinositol glycan anchor biosynthesis class A; phosphatidylinositol glycan anchor biosynthesis, class A (paroxysmal nocturnal
分子量： 54kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PIGA
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse,
细胞定位：细胞浆 细胞膜
红细胞磷脂酰肌醇聚糖A抗体产品介绍：background: PIGA belongs to the glycosyltransferase 1 family and is necessary for the synthesis of N-acetylglucosaminyl-phosphatidylinositol, the very early intermediate in GPI-anchor biosynthesis. Defects in PIGA are the cause of paroxysmal nocturnal hemoglobinuria (PNH) which is an acquired hemolytic blood disorder characterized by chronic hemolysis with hemoglobinuria, increased tendency to venous thrombosis, and variable degrees of bone marrow failure. Function: Necessary for the synthesis of N-acetylglucosaminyl-phosphatidylinositol, the very early intermediate in GPI-anchor biosynthesis. Subunit: Associates with PIGC, PIGH, PIGP, PIGQ 红细胞磷脂酰肌醇聚糖A抗体and DPM2. The latter is not essential for activity. Interacts directly with PIGY. Subcellular Location: Endoplasmic reticulum membrane; Single-pass membrane protein DISEASE: Defects in PIGA are the cause of paroxysmal nocturnal hemoglobinuria (PNH) [MIM:300818]. PNH is a disorder characterized by hemolytic anemia with hemoglobinuria, thromboses in large vessels, and a deficiency in hematopoiesis. Clinical manifestation of red blood cell breakdown with release of hemoglobin into the urine is manifested most prominently by dark-colored urine in红细胞磷脂酰肌醇聚糖A抗体 the morning. Similarity: Belongs to the glycosyltransferase group 1 family. Glycosyltransferase 4 subfamily. Database links: Entrez Gene: 5277 Human Entrez Gene: 18700 Mouse Omim: 311770 Human SwissProt: P37287 Human SwissProt: Q64323 Mouse Unigene: 137154 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
红细胞磷脂酰肌醇聚糖A抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:50-200 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：心血管 细胞生物
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid