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GAA 抗体 (Middle Region)

GAA 适用: 人, 大鼠 WB, IHC (p) 宿主: 兔 Polyclonal unconjugated
产品编号 ABIN4886596
发货至: 中国
  • 抗原 See all GAA 抗体
    GAA (Glucosidase, Alpha, Acid (GAA))
    抗原表位
    • 15
    • 9
    • 6
    • 6
    • 4
    • 3
    • 3
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 494-527, Middle Region
    适用
    • 45
    • 21
    • 19
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    人, 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
    • 15
    • 13
    • 13
    • 7
    • 7
    • 5
    • 3
    • 2
    • 2
    • 1
    Western Blotting (WB), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
    原理
    Rabbit IgG polyclonal antibody for Lysosomal alpha-glucosidase(GAA) detection. Tested with WB, IHC-P in Human,Rat.
    序列
    TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR
    交叉反应 (详细)
    No cross reactivity with other proteins.
    产品特性
    Rabbit IgG polyclonal antibody for Lysosomal alpha-glucosidase(GAA) detection. Tested with WB, IHC-P in Human,Rat.
    Gene Name: glucosidase alpha, acid
    Protein Name: Lysosomal alpha-glucosidase
    纯化方法
    Immunogen affinity purified.
    免疫原
    A synthetic peptide corresponding to a sequence in the middle region of human GAA (494-527aa TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR), different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.
    亚型
    IgG
    Top Product
    Discover our top product GAA Primary Antibody
  • 应用备注
    WB: Concentration: 0.1-0.5 μg/mL, Tested Species: Human, Rat
    IHC-P: Concentration: 0.5-1 μg/mL, Tested Species: Human, Epitope Retrieval by Heat: Boiling the paraffin sections in 10 mM citrate buffer, pH 6.0, for 20 mins is required for the staining of formalin/paraffin sections.
    Notes: Tested Species: Species with positive results. Other applications have not been tested. Optimal dilutions should be determined by end users.
    说明

    Antibody can be supported by chemiluminescence kit ABIN921124 in WB, supported by ABIN921231 in IHC(P).

    限制
    仅限研究用
  • 状态
    Lyophilized
    溶解方式
    Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
    浓度
    500 μg/mL
    缓冲液
    Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.05 mg Sodium azide.
    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.
    注意事项
    Avoid repeated freezing and thawing.
    储存条件
    4 °C/-20 °C
    储存方法
    At -20°C for one year. After reconstitution, at 4°C for one month.
    It can also be aliquotted and stored frozen at -20 °C for a longer time. Avoid repeated freezing and thawing.
  • 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
    Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene. 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.

    Synonyms: Acid alpha glucosidase | Acid maltase | Aglucosidase alfa | Alpha glucosidase | GAA | Glucosidase alpha acid | Glucosidase alpha | LYAG | P10253
    基因ID
    2548
    UniProt
    P10253
    途径
    Cellular Glucan Metabolic Process
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