| 產(chǎn)品詳情 |
| Edit |   |
| Product Name | Galactosidase, alpha (GLA) (AA 32-429) protein (His tag) |
| Description | alpha-Galactosidase A is a homodimeric glycoprotein that belongs to the glycosyl hydrolase 27 family. It is a lysosomal enzyme and used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. alpha-Galactosidase A can hydrolyze terminal alpha-galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose. Defects alpha-Galactosidase A are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease with 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. 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.Alternative Names: Alpha-Galactosidase A, Alpha-D-Galactosidase A, Alpha-D-Galactoside Galactohydrolase, Melibiase, Agalsidase, GLA |
| Size | 500 μg |
| Concentration | n/a |
| Applications | n/a |
| Other Names | MGC130872|SMU.877|SCF11.21|AO090005000217|GALA|zgc:101584|Ags|alpha-GAL|AW555139|GM130|mKIAA4150 |
| Gene, Accession, CAS # | Gene ID: 2717 |
| Catalog # | ABIN1691084 |
| Price | |
| Order / More Info | Galactosidase, alpha (GLA) (AA 32-429) protein (His tag) from ANTIBODIES-ONLINE GmbH |
| Product Specific References | n/a |
| 產(chǎn)品資料 |
|
日韩欧美一区二区三区久久精品,国产日韩在线一区,肏屄黄片
|
日本不卡视频在线,久久天天综合桃花久久,777午夜
|
欧美老妇性猛交XXXX免费看,乌克兰少妇xxxx做受,五月天激情快乐网
|
天天干天天添,国产黄色网,玖玖一区
|
小视频操逼,女人18片毛片90分钟免费,豆花视频官方网站免费
|
久久网中文字幕,无遮挡国产高潮视频免费观看,插女人的视频18
|
国产福利一区二区三区视频,一区二区网站,91丨国产丨熟女 熟女
|
国产一级无码,国产免费拔擦拔擦8x在线下载,h网站在线
|
啪啪免费网,主人好湿好紧太爽了,亚洲色婷婷影视
|
在线操逼,一区视频在线播放,九一精品视频
|
|