SAB5702125

Anti-GAA Antibody, clone 5L2L10, Rabbit Monoclonal

• 别名: GAA, LYAG, glucosidase alpha, acid
• 分類程式碼代碼: 12352203

属性

• 生物源: rabbit
• 品質等級: 100
• 材料: colorless
• 無性繁殖: 5L2L10, monoclonal
• 形狀: liquid
• 物種活性: rat, rat, human
• 濃度: 0.8 mg/mL
• 技術: western blot: 1:500 - 1:2000
• 顏色: colorless
• 同型: IgG
• 免疫原序列: QEQCEARGCCYIPAKQGLQGAQMGQPWCFFPPSYPSYKLENLSSSEMGYTATLTRTTPTFFPKDILTLRLDVMMETENRLHFTIKDPANRRYEVPLETPHV
• 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

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.

應用

WB

安全信息

• 儲存類別代碼: 10 - Combustible liquids
• 水污染物質分類（WGK）: WGK 1