生物来源
rabbit
质量水平
物料
colorless
克隆
7G8T9, monoclonal
表单
liquid
分子量
50 kDa
种属反应性
human, rat, rat
浓度
1.23mg/mL
技术
western blot: 1:500 - 1:2000
颜色
colorless
同位素/亚型
IgG
免疫原序列
FMCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWMAPQRDSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFG
UniProt登记号
运输
wet ice
储存温度
−20°C
靶向翻译后修饰
unmodified
基因信息
human ... GLA(2717)
一般描述
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]
免疫原
A synthetic peptide corresponding to a sequence within amino acids 50-150 of human Galactosidase alpha (GLA) (GLA) (P06280).
PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
应用
WB
储存分类代码
10 - Combustible liquids
WGK
WGK 1
法规信息
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