GAA 抗体 (Middle Region) (Cy3)
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Quick Overview for GAA 抗体 (Middle Region) (Cy3) (ABIN7993500)
抗原
See all GAA 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- Middle Region
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原理
- Anti-GAA Antibody Cy3 Conjugated
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特异性
- No cross reactivity with other proteins.
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交叉反应 (详细)
- No cross-reactivity with other proteins
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预测反应
- different from the related mouse sequence by eight amino acids,and from the related rat sequence by six amino acids.
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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
- IgG
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应用备注
- Flow Cytometry, 1-3 μg/1x106 cells
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限制
- 仅限研究用
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状态
- Liquid
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缓冲液
- Each vial contains 50 % glycerol, 0.9 % NaCl, 0.2 % Na2HPO4, 0.02 % 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
- -20 °C
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储存方法
- At -20°C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.
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有效期
- 12 months
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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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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.
Gene Full Name: alpha glucosidase
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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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