Anti-GLA / alpha-Galactosidase A Antibody

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Anti-GLA / alpha-Galactosidase A Antibody (Rabbit Monoclonal 抗体) 产品信息

产品名称
Anti-GLA / alpha-Galactosidase A Antibody
经验证的应用
WB,ELISA,IP
交叉反应
Reacts with: Human
特异性
Human GLA / alpha-Galactosidase A
免疫原
Recombinant Human alpha-Galactosidase A / GLA protein (Catalog#12078-H08H)
制备方法
This antibody was obtained from a rabbit immunized with purified, recombinant Human alpha-Galactosidase A / GLA (rh alpha-Galactosidase A / GLA; Catalog#12078-H08H; AAP36507.1; Met 1-Leu 429).
来源
Monoclonal Rabbit IgG Clone #001
纯化
Protein A
缓冲液
0.2 μm filtered solution in PBS
偶联物
Unconjugated
状态
Liquid
运输方式
This antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
储存条件
This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free. Avoid repeated freeze-thaw cycles.

Anti-GLA / alpha-Galactosidase A Antibody (Rabbit Monoclonal 抗体) 经验证的应用

应用 推荐稀释比/用量
WB 1:1000-1:5000
ELISA 1:5000-1:10000
IP 4-6 μL/mg of lysate
Please Note: Optimal concentrations/dilutions should be determined by the end user.

Anti-GLA / alpha-Galactosidase A Antibody (Rabbit Monoclonal 抗体) 图片

Anti-GLA rabbit monoclonal antibody at 1:1000 dilution

Lane A: 293T Whole Cell Lysate

Lysates/proteins at 30 μg per lane.

Secondary

Goat Anti-Rabbit IgG H&L (Dylight800) at 1/10000 dilution.

Developed using the Odyssey technique.

Performed under reducing conditions.

Predicted band size:49 kDa

Observed band size:49 kDa

GLA was immunoprecipitated using:

Lane A:0.5 mg MCF-7 Whole Cell Lysate

2 µL anti-GLA rabbit monoclonal antibody and 15 μl of 50 % Protein G agarose.

Primary antibody:

Anti-GLA rabbit monoclonal antibody,at 1:1000 dilution

Secondary antibody:

Clean-Blotô IP Detection Reagent (HRP) at 1:1000 dilution

Developed using the DAB staining technique.

Performed under reducing conditions.

Predicted band size: 49 kDa

Observed band size: 52 kDa

Anti-GLA / alpha-Galactosidase A Antibody: 别称

Anti-GALA Antibody

GLA / alpha-Galactosidase A 背景信息

Alpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction.
全称
galactosidase, alpha
参考文献
  • Koide T.et al., 1990, FEBS Lett. 259:353-356.
  • Yang C.-C. et al., 2003, Clin. Genet. 63:205-209.
  • Verovnik F. et al.,2004, Eur. J. Hum. Genet. 12:678-681.
  • Nance C.S. et al., 2006, Arch. Neurol. 63:453-457.
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