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货号： bs-9524R-Cy3 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-9524R-Cy3
- 英文名称
- Anti-PIGA/Cy3
- 中文名称
- Cy3标记的红细胞磷脂酰肌醇聚糖A抗体
- 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 hemoglobinuria); Phosphatidylinositol N-acetylglucosaminyltransferase subunit A; PIG A; PIG-A; PIGA_HUMAN; Phosphatidylinositol-glycan biosynthesis class A protein; PIG-A.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 心血管 细胞生物
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse,
- 产品应用
- IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 54kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PIGA
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
- 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 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 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.