PIGA,红细胞磷脂酰肌醇聚糖A抗体

产品编号HZ-9524R
英文名称PIGA
中文名称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.
说 明 书0.1ml 0.2ml
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,
PIGA,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.
分 子 量54kDa
细胞定位细胞浆 细胞膜
性 状Lyophilized or Liquid
浓 度1mg/1ml
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.
PIGA,红细胞磷脂酰肌醇聚糖A抗体PubMedPubMed
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.

PIGA,红细胞磷脂酰肌醇聚糖A抗体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.