商品属性：
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产品名称
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HEXA抗体
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英文名称
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HEXA
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产品规格
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50ul 100ul 200ul
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产品货号
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GOY-01K1091
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产品分类
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抗体 / 一抗
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交叉反应
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Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep
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保存条件
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Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
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标记
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Unconjugated
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抗体来源
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Rabbit
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抗体类型
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Polyclonal
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研究领域
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细胞生物 神经生物学
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蛋白细胞定位
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细胞浆
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纯化方法
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affinity purified by Protein A
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性状
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Liquid
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浓度
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1mg/ml
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储存液
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0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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发货地
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上海
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背景资料
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Hexosaminidase A (HEXA), also designated beta-Hexosaminidase A, is a trimer composed of one ? chain, one ∫-A chain and one ∫-B chain and is found in the lysosomes of cells. HEXA, along with the cofactor CM2 activator protein, catalyzes the degradation of GM2 ganglioside and other molecules containing terminal N-acetyl hexosamines in the brain and other tissues. A mutation in the ? subunit of hexosaminidase is the cause of Tay-Sachs disease (TSD), also known as GM2-gangliosidosis type I. TSD is a fatal autosomal recessive lysosomal storage disease of the central nervous system (CNS) caused by insufficient activity of the HEXA enzyme that results in a failure to process GM2 gangliosides. The accumulation of GM2 ganglioside in the absence of HEXA activity causes progressive destruction of the CNS
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