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GAA 抗体 (AA 541-640)

GAA 适用: 人, 小鼠, 大鼠 WB, ELISA, ICC, IF (cc), IF (p), IHC (p), IHC (fro) 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN1714619
发货至: 中国
  • 抗原 See all GAA 抗体
    GAA (Glucosidase, Alpha, Acid (GAA))
    抗原表位
    • 14
    • 9
    • 6
    • 6
    • 4
    • 3
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 541-640
    适用
    • 45
    • 20
    • 19
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    人, 小鼠, 大鼠
    宿主
    • 45
    • 4
    克隆类型
    • 46
    • 3
    多克隆
    标记
    • 24
    • 5
    • 4
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    This GAA antibody is un-conjugated
    应用范围
    • 45
    • 19
    • 14
    • 12
    • 12
    • 7
    • 6
    • 5
    • 2
    • 2
    • 2
    • 1
    Western Blotting (WB), ELISA, Immunocytochemistry (ICC), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p)), Immunohistochemistry (Frozen Sections) (IHC (fro))
    交叉反应
    人, 小鼠, 大鼠
    预测反应
    Dog,Sheep,Pig,Horse
    纯化方法
    Purified by Protein A.
    免疫原
    KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha
    亚型
    IgG
    Top Product
    Discover our top product GAA Primary Antibody
  • 应用备注
    WB 1:300-5000
    ELISA 1:500-1000
    IHC-P 1:200-400
    IHC-F 1:100-500
    IF(IHC-P) 1:50-200
    IF(IHC-F) 1:50-200
    IF(ICC) 1:50-200
    ICC 1:100-500
    限制
    仅限研究用
  • 状态
    Liquid
    浓度
    1 μg/μL
    缓冲液
    0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
    储存液
    ProClin
    注意事项
    This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
    储存条件
    4 °C,-20 °C
    储存方法
    Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
    有效期
    12 months
  • 抗原
    GAA (Glucosidase, Alpha, Acid (GAA))
    别名
    GAA (GAA 产品)
    别名
    LYAG antibody, E430018M07Rik antibody, glucosidase alpha, acid antibody, glucosidase, alpha, acid antibody, transmembrane and coiled-coil domain family 1 antibody, glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II) antibody, GAA antibody, Gaa antibody, TMCC1 antibody, gaa antibody
    背景

    Synonyms: 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.

    Background: 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].

    基因ID
    2548
    UniProt
    P10253
    途径
    Cellular Glucan Metabolic Process
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