GAA 抗体
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Quick Overview for GAA 抗体 (ABIN7880880)
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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原理
- Glucosidase Alpha Acid Antibody / GAA
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序列
- TALAWWEDMV AEFHDQVPFD GMWIDMNEPS NFIR
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纯化方法
- Affinity purified
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免疫原
- Amino acids TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR from the human protein were used as the immunogen for the Glucosidase Alpha Acid antibody.
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亚型
- IgG2b
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anti-Glucosidase, Alpha, Acid (GAA) (AA 174-203), (N-Term) antibody
GAA 适用: 人 WB, IHC, IHC (p) 宿主: 兔 Polyclonal RB18979 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 595-770) antibodyGAA 适用: 人 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 851-952) antibodyGAA 适用: 人 WB, ELISA 宿主: 小鼠 Monoclonal 3C6 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 174-203) antibodyGAA 适用: 人 WB, IHC, IHC (p) 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, ICC, ELISA, 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
- Optimal dilution of the Glucosidase Alpha Acid antibody should be determined by the researcher.
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限制
- 仅限研究用
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状态
- Lyophilized
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缓冲液
- 0.5 mg/mL if reconstituted with 0.2 mL sterile DI water
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储存条件
- 4 °C,-20 °C
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储存方法
- After reconstitution, the Glucosidase Alpha Acid antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
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- GAA (Glucosidase, Alpha, Acid (GAA))
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别名
- Glucosidase Alpha Acid
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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.
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UniProt
- P10253
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途径
- Cellular Glucan Metabolic Process
抗原
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