Galactosidase alpha (GLA) Rabbit mAb (AMR13138N)
产品名称: Galactosidase alpha (GLA) Rabbit mAb (AMR13138N)
英文名称: Galactosidase alpha (GLA) Rabbit mAb (AMR13138N)
产品编号: AMR13138N
产品价格: null
产品产地: 美国
品牌商标: Leading Biology
更新时间: null
使用范围:
安诺伦(北京)生物科技有限公司
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- 地址 : 北京大兴经济技术开发区天骥智谷35A
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分子量:Calculated MW: 46kDa Observed MW: 50KDa 细胞定位:Lysosome 稀释方法:WB 1:500 - 1:2000 总结:This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. [provided by RefSeq, Jul 2008]