SAB2100872

抗GAA ウサギ宿主抗体

affinity isolated antibody

• 別名: 抗LYAG抗体, 抗酸性α-グルコシダーゼ抗体
• UNSPSCコード: 12352203
• NACRES: NA.41

特性

• 由来生物: rabbit
• 品質水準: 100
• 結合体: unconjugated
• 抗体製品の状態: affinity isolated antibody
• 抗体製品タイプ: primary antibodies
• クローン: polyclonal
• 形状: buffered aqueous solution
• 分子量: 98 kDa
• 化学種の反応性: dog, guinea pig, human, rat, mouse, bovine
• 濃度: 0.5 mg - 1 mg/mL
• テクニック: immunohistochemistry: suitable; western blot: suitable
• UniProtアクセッション番号: P10253
• 輸送温度: wet ice
• 保管温度: −20°C
• ターゲットの翻訳後修飾: unmodified
• 遺伝子情報: human ... GAA(2548)

詳細

詳細

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

アプリケーション

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

生物化学的／生理学的作用

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.

シーケンス

Synthetic peptide located within the following region: FGVIVRRQLDGRVLLNTTVAPLFFADQFLQLSTSLPSQYITGLAEHLSPL

物理的形状

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

免責事項

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.

安全性情報

• 保管分類コード: 10 - Combustible liquids
• WGK: WGK 3
• 引火点(°F): Not applicable
• 引火点(℃): Not applicable