Anti-GAA antibody produced in rabbit

半岛bd体育手机客户端 说明

一般描述

Lysosomal alpha-glucosidase (GAA) is a glycoprotein that belongs to the glycoside hydrolase family GH31. It comprises a catalytic GH31 (β/α)8 barrel domain, trefoil type-P domain, an N-terminal β-sheet domain. The GAA gene is mapped to human chromosome location 17q25.3.

免疫原

Synthetic peptide directed towards the N terminal region of human GAA

应用

Anti-GAA antibody produced in rabbit has been used western blotting (1:100)

生化/生理作用

GAA is acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene.

序列

Synthetic peptide located within the following region: FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL

外形

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

免责声明

Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

基本信息

NACRES

NA.41

半岛bd体育手机客户端 性质

生物来源	rabbit
质量水平	100
偶联物	unconjugated
抗体形式	affinity isolated antibody
antibody product type	primary antibodies
克隆	polyclonal
形式	buffered aqueous solution
分子量	98 kDa
species reactivity	mouse, dog, bovine, human, rat, guinea pig
浓度	0.5 mg - 1 mg/mL
technique(s)	immunohistochemistry: suitable western blot: suitable
UniProt登记号	P10253
运输	wet ice
储存温度	?20℃
Gene Information	human ... GAA(2548)

安全信息

储存分类代码	12 - Non Combustible Liquids
WGK	WGK 3
闪点(F)	Not applicable
闪点(C)	Not applicable