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HEXA rabbit pAb
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ES3963
规格： 价格：
50µL ￥1280.00
100µL ￥1980.00
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Overview

产品名称: HEXA rabbit pAb
反应性: Human;Mouse;Rat
别名: HEXA; Beta-hexosaminidase subunit alpha; Beta-N-acetylhexosaminidase subunit alpha; Hexosaminidase subunit A; N-acetyl-beta-glucosaminidase subunit alpha
宿主: Rabbit
稀释比: Western Blot: 1/500 - 1/2000. IHC-p: 1:100-300 ELISA: 1/20000. Not yet tested in other applications.
免疫原: Synthesized peptide derived from HEXA . at AA range: 121-170
储存条件: -20°C/1 year
克隆性: Polyclonal
抗体亚型: IgG
浓度: 1 mg/ml
实测条带: 60kD
基因ID: 3073
蛋白序列: P06865
细胞定位: Lysosome.
研究背景: This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I). Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. [provided by RefSeq, Jan 2016],
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