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Anti-GLA/alpha-Galactosidase A Antibody, Rabbit Polyclonal
Anti-GLA/alpha-Galactosidase A Antibody, Rabbit Polyclonal
- 中文名称：
- Anti-GLA/alpha-Galactosidase A Antibody, Rabbit Polyclonal
- 英文名称：
- Anti-GLA/alpha-Galactosidase A Antibody, Rabbit Polyclonal
- 品牌：
- AAA Biotech
- 品牌介绍：
- AAA Biotech专注于为全球生命科学研究提供高品质的蛋白质研究工具，核心产品包括经严格验证的抗体、重组蛋白及ELISA试剂盒。
- 货号：
- AAA259429
- 规格：
- 0.1 mL|2x0.1 mL|3x0.1 mL|4x0.1 mL|5x0.1 mL
- 保存建议：
- This antibody can be stored at 2 degree C-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Preservative-Free. Avoid repeated freeze-thaw cycles.
This antibody is shipped as liquid solution at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.
- 货期：
- 6-8周
- 来源宿主：
- Rabbit
- 反应种属：
- Human
- 应用：
- IP (Immunoprecipitation), ELISA, WB (Western Blot)
- 免责声明：
- *本产品仅供科研实验使用，不得用于临床诊断。*
- 其他：
克隆性:Polyclonal
同型:Rabbit IgG
克隆号:N/A
特异性:Human GLA/alpha-Galactosidase A
纯度:Protein A & Antigen Affinity
形式：Liquid; 0.2um filtered solution in PBS
浓度：N/A
- 说明书：
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 identification 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.
