SAB5702125

Anti-GAA Antibody, clone 5L2L10, Rabbit Monoclonal

• 別名: GAA, LYAG, glucosidase alpha, acid
• UNSPSCコード: 12352203

特性

• 由来生物: rabbit
• 品質水準: 100
• 物質: colorless
• クローン: 5L2L10, monoclonal
• 形状: liquid
• 化学種の反応性: rat, rat, human
• 濃度: 0.8 mg/mL
• テクニック: western blot: 1:500 - 1:2000
• 色: colorless
• アイソタイプ: IgG
• 免疫原配列: QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV
• 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

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.

アプリケーション

WB

安全性情報

• 保管分類コード: 10 - Combustible liquids
• WGK: WGK 1