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生物来源
rabbit
质量水平
物料
colorless
克隆
5L2L10, monoclonal
表单
liquid
种属反应性
rat, rat, human
浓度
0.8 mg/mL
技术
western blot: 1:500 - 1:2000
颜色
colorless
同位素/亚型
IgG
免疫原序列
QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV
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 ... GAA(2548)
一般描述
This gene encodes lysosomal alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme. Defects in this gene are the cause of glycogen storage disease II, also known as Pompe′s disease, which is an autosomal recessive disorder with a broad clinical spectrum. Alternative splicing results in multiple transcript variants.
免疫原
A synthetic peptide corresponding to a sequence within amino acids 100-200 of human GAA (P10253).
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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