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折扣/货期| 货号：GTX03539-pro | 规格：100μg | 目录价：￥6100 |
产品详情
产品名称:
Human HEXB protein, His tag (active)
别名:
hexosaminidase subunit beta , ENC-1AS , HEL-248 , HEL-S-111
实验应用:
Functional Assay
克隆号:
偶联:
Unconjugated
产品浓度:
表达系统:
HEK293 Cells
保存温度:
Store at -20ºC or below. After reconstitution, keep as concentrated solution. Aliquot and store at -20ºC or below. Avoid freeze-thaw cycles.
运输温度:
4°C
产品形式:
Lyophilized powder
存储溶液:
Batch dependent (please contact us for details)
产地:
美国
功能与背景:
Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioseside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioseside in neurons and neurodegenerative disorders termed the GM2 gangliosesidoses. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosesidosis type II). Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2014]
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