中文 | English
产品分类
·瑞舒伐他汀钙及中间体
·苯磺顺阿曲库铵及中间体
·原料药
·中间体
·Diagnostic & Assay Kits
·标准品
·固化剂
·化合物
产品搜索
联系我们
地址:南京市江宁区天印大道天印慧谷3号商务楼1323
手机:15895913691
电话:+86-25-52719751
传真:+86-25-52719785
客服QQ:2630196394   1839208651
邮箱:admin@chemdiate.com
产品中心  
Alpha-galactosidase assay
Alpha-galactosidase-A hydrolyzes the terminal alpha-galactosyl moieties from glycolipids and glycoproteins in lysosomes. Mutations in α-galactosidase cause lysosomal accumulation of the glycosphingolipid, globotriaosylceramide, which leads to Fabry disease.  In males, the most efficient and reliable method for confirmation of the diagnosis is the demonstration of deficient alpha-galactosidase A enzyme activity in plasma, isolated leukocytes, and/or cultured cells. The test is a fluorometric assay using the alternative substrate 4-methylumbelliferyl-alpha-D-galactopyranoside. Males with classic Fabry disease have less than 1% alpha-galactosidase A activity. 
Chemediate provides assay kit for detecting Alpha-galactosidase activity in either the purified form or biological samples. Pretreatment of samples are not required. Alpha-galactosidase activity can be measured using fluorometer (Ex/Em = 360/440 nm).
Assay Principle:
Copyright 2012-2021 www.chemdiate.com All rights reserved
南京蓝碧安生化科技有限公司 版权所有 苏ICP备12031533号-1 技术支持:南京做网站
QQ客服