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GAA Antibody Blocking Peptide(

bs-13254P)-500ug
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  • 2025年10月16日
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      500ug

    产品编号bs-13254P
    英文名称GAA Antibody Blocking Peptide
    中文名称α葡萄糖苷酶/溶酶体α-葡糖苷酶封闭多肽
    英文别名70 kDa lysosomal alpha-glucosidase; Acid alpha glucosidase; Acid maltase; Aglucosidase alfa; Alpha glucosidase; GAA; Glucosidase alpha acid (Pompe disease glycogen storage disease type II); Glucosidase alpha acid; Glucosidase alpha; LYAG; LYAG_HUMAN; Lysosomal alpha glucosidase.
    纯化方法HPLC
    研究领域

    Metabolism > Pathways and Processes > Metabolic signaling pathways > Energy transfer pathways > Energy Metabolism

    Signal Transduction > Metabolism > Energy Metabolism

    亚细胞定位Lysosome. Lysosome membrane.
    翻译后修饰The different forms of acid glucosidase are obtained by proteolytic processing.
    Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor.
    相似性Belongs to the glycosyl hydrolase 31 family.
    Contains 1 P-type (trefoil) domain.
    功能Essential for the degradation of glygogen to glucose in lysosomes. Subcellular Location : Lysosome. Lysosome membrane.
    保存条件Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles.
    注意事项This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
    背景资料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. [provided by RefSeq, Jul 2008].

     

     

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