SAB2100872

Anti-GAA antibody produced in rabbit

affinity isolated antibody

• 别名: Anti-Glucosidase, α; acid, Anti-LYAG
• 分類程式碼代碼: 12352203
• NACRES: NA.41

属性

• 生物源: rabbit
• 品質等級: 100
• 共軛: unconjugated
• 抗體表格: affinity isolated antibody
• 抗體產品種類: primary antibodies
• 無性繁殖: polyclonal
• 形狀: buffered aqueous solution
• 分子量: 98 kDa
• 物種活性: dog, guinea pig, human, rat, mouse, bovine
• 濃度: 0.5 mg - 1 mg/mL
• 技術: immunohistochemistry: suitable; western blot: suitable
• UniProt登錄號: P10253
• 運輸包裝: wet ice
• 儲存溫度: −20°C
• 目標翻譯後修改: unmodified
• 基因資訊: human ... GAA(2548)

说明

一般說明

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

應用

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

外觀

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

免責聲明

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.

安全信息

• 儲存類別代碼: 10 - Combustible liquids
• 水污染物質分類（WGK）: WGK 3
• 閃點（°F）: Not applicable
• 閃點（°C）: Not applicable