WH0002548M1

Monoclonal Anti-GAA antibody produced in mouse

clone 3C6, purified immunoglobulin, buffered aqueous solution

• 同義詞: Anti-LYAG, Anti-glucosidase, α acid (Pompe disease, glycogen storage disease type II)
• 分類程式碼代碼: 12352203
• NACRES: NA.41

屬性

• 生物源: mouse
• 品質等級: 100
• 共軛: unconjugated
• 抗體表格: purified immunoglobulin
• 抗體產品種類: primary antibodies
• 無性繁殖: 3C6, monoclonal
• 形狀: buffered aqueous solution
• 物種活性: human
• 技術: indirect ELISA: suitable; western blot: 1-5 μg/mL
• 同型: IgG1κ
• GenBank登錄號: BC040431
• UniProt登錄號: P10253
• 運輸包裝: dry ice
• 儲存溫度: −20°C
• 目標翻譯後修改: unmodified
• 基因資訊: human ... GAA(2548)

描述

一般說明

Lysosomal α-glucosidase (GAA), a glycoprotein and member of glycoside hydrolase family GH31, comprises a trefoil type-P domain, catalytic GH31 domain, distal, proximal, and an N-terminal β-sheet domain. The GAA gene is mapped to human chromosome 17q25.3. It undergoes various proteolytical and N-glycan processing in the late endosomal/lysosomal compartment to become an active form.

免疫原

GAA (AAH40431, 851 a.a. ~ 952 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Sequence
GEARGELFWDDGESLEVLERGAYTQVIFLARNNTIVNELVRVTSEGAGLQLQKVTVLGVATAPQQVLSNGVPVSNFTYSPDTKVLDICVSLLMGEQFLVSWC

生化／生理作用

Lysosomal α-glucosidase (GAA) uses mannose-6-phosphate receptors for its localization on the lysosomes. It mediates the hydrolysis of glycogen to glucose. Mutations in the GAA gene impair acid alpha-glucosidase enzyme activity. Deficiency of GAA leads to a rare lysosomal storage disease namely Pompe disease.

外觀

Solution in phosphate buffered saline, pH 7.4

法律資訊

GenBank is a registered trademark of United States Department of Health and Human Services

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.

安全資訊

• 儲存類別代碼: 10 - Combustible liquids
• 閃點（°F）: Not applicable
• 閃點（°C）: Not applicable
• 個人防護裝備: Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)