电话:
+86 (0512) 65829739
传真:
+86 (010) 6788 5057
电子邮件:
orders@antibodies-online.cn

GAA 抗体 (Middle Region)

This anti-GAA antibody is a 兔 多克隆 antibody detecting GAA in WB 和 IHC. Suitable for 人.
产品编号 ABIN4886596
发货至: 中国

Quick Overview for GAA 抗体 (Middle Region) (ABIN4886596)

抗原

See all GAA 抗体
GAA (Glucosidase, Alpha, Acid (GAA))

适用

  • 50
  • 25
  • 23
  • 2
  • 2
  • 2
  • 2
  • 1
  • 1
  • 1
  • 1
  • 1

宿主

  • 54
  • 6

克隆类型

  • 52
  • 8
多克隆

标记

  • 40
  • 4
  • 3
  • 2
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
  • 1
This GAA antibody is un-conjugated

应用范围

  • 54
  • 29
  • 18
  • 18
  • 14
  • 13
  • 13
  • 7
  • 4
  • 3
  • 2
  • 1
Western Blotting (WB), Immunohistochemistry (IHC)
  • 抗原表位

    • 15
    • 7
    • 4
    • 4
    • 4
    • 3
    • 3
    • 2
    • 2
    • 2
    • 2
    • 1
    • 1
    • 1
    • 1
    • 1
    • 1
    AA 494-527, Middle Region

    原理

    Anti-GAA Antibody Picoband®

    序列

    TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR

    交叉反应 (详细)

    No cross-reactivity with other proteins

    产品特性

    Anti-GAA Antibody Picoband® (ABIN4886596). Tested in IHC, WB applications. This antibody reacts with Human. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

    纯化方法

    Immunogen affinity purified.

    免疫原

    A synthetic peptide corresponding to a sequence in the middle region of human GAA, different from the related mouse sequence by eight amino acids, and from the related rat sequence by six amino acids.

    亚型

    IgG
  • 应用备注

    Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human
    Western blot, 0.1-0.5 μg/mL, Human
    1. "Entrez Gene: GAA glucosidase, alpha, acid (Pompe disease, glycogen storage disease type II)". 2. Donald J. Voet, Judith G. Voet, Charlotte W. Pratt (2008). "Additional Pathways in Carbohydrate Metabolism". Principles of Biochemistry, Third edition. Wiley. p. 538. 3. Reuser AJ, Kroos MA, Hermans MM, et al. (1995). "Glycogenosis type II (acid maltase deficiency).". Muscle Nerve. 3: S61-9.

    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

    Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month.
    It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.
  • 抗原

    GAA (Glucosidase, Alpha, Acid (GAA))

    别名

    GAA

    背景

    Synonyms: Lysosomal alpha-glucosidase,3.2.1.20,Acid maltase,Aglucosidase alfa,76 kDa lysosomal alpha-glucosidase,70 kDa lysosomal alpha-glucosidase,GAA,

    Tissue Specificity: Most abundant in heart, brain, liver, skeletal muscle and testis but absent in thymus and peripheral blood leukocytes.

    Background: 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.

    分子量

    110 kDa, 95kDa, 76kDa, 70 kDa

    基因ID

    2548

    UniProt

    P10253

    途径

    Cellular Glucan Metabolic Process
You are here:
Chat with us!