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GAA 抗体

GAA 适用: 人, 小鼠, 大鼠 WB 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN7263675
发货至: 中国
  • 抗原 See all GAA 抗体
    GAA (Glucosidase, Alpha, Acid (GAA))
    适用
    • 47
    • 21
    • 20
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    人, 小鼠, 大鼠
    宿主
    • 47
    • 4
    克隆类型
    • 47
    • 4
    多克隆
    标记
    • 26
    • 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
    • 47
    • 19
    • 15
    • 13
    • 13
    • 8
    • 7
    • 5
    • 3
    • 2
    • 2
    • 1
    Western Blotting (WB)
    产品特性
    Polyclonal Antibody
    纯化方法
    Affinity purification
    免疫原
    A synthetic peptide of human GAA (NP_000143.2).
    亚型
    IgG
    Top Product
    Discover our top product GAA Primary Antibody
  • 应用备注
    WB 1:500-1:2000
    限制
    仅限研究用
  • 状态
    Liquid
    浓度
    1 mg/mL
    缓冲液
    PBS with 0.02 % sodium azide, 50 % glycerol, pH 7.3
    储存液
    Sodium azide
    注意事项
    This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
    储存条件
    -20 °C
    储存方法
    Store at -20°C. Avoid freeze / thaw cycles.
  • 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
    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.
    分子量

    Observed_MW: 105 kDa

    Calculated_MW: 105 kDa

    基因ID
    2548
    UniProt
    P10253
    途径
    Cellular Glucan Metabolic Process
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