别称
Alpha galactosidase A, galactosidase, alpha
宿主
Mouse
适应种属
Human
亚型
IgG2a
验证应用
ELISA, WB, IHC, IF
产品信息
产品名称
anti- Alpha galactosidase A antibody
货号
QSab00330
规格
100μg
状态
liquid
纯化方法
Protein A+G purification
纯度
≥95% as determined by SDS-PAGE
宿主
Mouse
克隆性
monoclonal
亚型
IgG2a
存储
PBS with 0.02% sodium azide and 50% glycerol pH 7.3,-20℃ for 12 months(Avoid repeated freeze / thaw cycles.)
背景介绍
GLA, also named as Melibiase, Agalsidase and Alpha-galactosidase A, belongs to the glycosyl hydrolase 27 family. It hydrolyzes terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids. Fabry disease is an X-linked lysosomal storage disorder resulting from the deficient activity of GLA. Enzyme replacement therapy (ERT) with GLA is currently the most effective therapeutic strategy for patients with Fabry disease, a lysosomal storage disease.
免疫原信息
免疫原
galactosidase, alpha
别称
Alpha galactosidase A, galactosidase, alpha
分子量
49 kDa
应用
验证应用
ELISA, WB, IHC, IF
建议稀释比例
WB :1:500-1:2000; IHC: 1:20-1:200; IF: 1:20-1:200