GAA 抗体 (Middle Region)
Quick Overview for GAA 抗体 (Middle Region) (ABIN7602983)
抗原
See all GAA 抗体适用
宿主
克隆类型
标记
应用范围
克隆位点
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抗原表位
- Middle Region
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原理
- Anti-GAA Antibody Picoband® (monoclonal, 2G7)
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交叉反应 （详细）
- No cross-reactivity with other proteins.
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产品特性
- Anti-GAA Antibody Picoband® (monoclonal, 2G7) (ABIN7602983). Tested in IF, IHC, ICC, 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.
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纯化方法
- Immunogen affinity purified.
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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.
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亚型
- IgG2b
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anti-Glucosidase, Alpha, Acid (GAA) (AA 595-770) antibody
GAA 适用: 人 WB, IHC, IP, ICC 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Alpha, Acid (GAA) (AA 755-953) antibodyGAA 适用: 小鼠 WB, IHC, IP, ICC 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Alpha, Acid (GAA) (AA 601-952) antibodyGAA 适用: 人 WB, IHC, ELISA, IF 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Alpha, Acid (GAA) (AA 174-203), (N-Term) antibodyGAA 适用: 人 WB, IHC, IHC (p) 宿主: 兔 Polyclonal RB18979 unconjugated
anti-Glucosidase, Alpha, Acid (GAA) (AA 851-952) antibodyGAA 适用: 人 WB, ELISA 宿主: 小鼠 Monoclonal 3C6 unconjugated
anti-Glucosidase, Alpha, Acid (GAA) (AA 174-203) antibodyGAA 适用: 人 WB, IHC, IHC (p) 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Alpha, Acid (GAA) (AA 1-952) antibodyGAA 适用: 人 WB 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Alpha, Acid (GAA) (AA 494-527), (Middle Region) antibodyGAA 适用: 人 WB, IHC 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Alpha, Acid (GAA) (AA 541-640) antibodyGAA 适用: 人, 小鼠, 大鼠 WB, ELISA, ICC, IF (cc), IF (p), IHC (p), IHC (fro) 宿主: 兔 Polyclonal unconjugated
anti-Glucosidase, Alpha, Acid (GAA) (AA 70-225) antibodyGAA 适用: 大鼠 WB, IHC, IP, ICC 宿主: 兔 Polyclonal unconjugated
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应用备注
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Western blot, 0.1-0.5 μg/mL, Human
Immunohistochemistry (Paraffin-embedded Section), 0.5-1 μg/mL, Human
Immunocytochemistry/Immunofluorescence, 2 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. -
限制
- 仅限研究用
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状态
- Lyophilized
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溶解方式
- Add 0.2 mL of distilled water will yield a concentration of 500 μg/mL.
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浓度
- 500 μg/mL
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缓冲液
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4, 0.01 mg Sodium azide.
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储存液
- Sodium azide
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注意事项
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- 4 °C,-20 °C
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储存方法
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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.
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- GAA (Glucosidase, Alpha, Acid (GAA))
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别名
- GAA
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背景
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Synonyms: Lysosomal alpha-glucosidase,3.2.1.20
Tissue Specificity: Expressed on platelets and leukocytes and is primarily concentrated at the borders between endothelial cells. Expressed in human umbilical vein endothelial cells (HUVECs) (at protein level). Expressed on neutrophils (at protein level). Isoform Long predominates in all tissues examined. Isoform Delta12 is detected only in trachea. Isoform Delta14-15 is only detected in lung. Isoform Delta14 is detected in all tissues examined with the strongest expression in heart. Isoform Delta15 is expressed in brain, testis, ovary, cell surface of platelets, human umbilical vein endothelial cells (HUVECs), Jurkat T-cell leukemia, human erythroleukemia (HEL) and U-937 histiocytic lymphoma cell lines (at protein level).
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.
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分子量
- 110 kDa, 95 kDa, 76 kDa
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基因ID
- 2548
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UniProt
- P10253
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途径
- Cellular Glucan Metabolic Process
抗原
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