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折扣/货期| 货号：GTX55224 | 规格：100μl | 目录价：￥4700 |
产品详情
* 以下信息仅供参考，详情请以原厂网站为准
产品名称:
HEXB antibody
别名:
hexosaminidase subunit beta , ENC-1AS , HEL-248 , HEL-S-111
反应种属:
Human
宿主来源:
Rabbit
实验应用:
ICC/IF, IHC-P, WB
同种型:
IgG
免疫原:
KLH-conjugated synthetic peptide encompassing a sequence within the C-term region of HEXB. The exact sequence is proprietary.
克隆性:
Polyclonal
克隆号:
纯化方式:
Purified by antigen-affinity chromatography
偶联:
Unconjugated
产品浓度:
Batch dependent (Please refer to the vial label for the specific concentration.)
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
预期分子量:
63
产品形式:
Liquid
存储溶液:
0.42% Potassium Phosphate, 0.87% NaCl, 30% Glycerol, 0.01% Sodium azide.
生产商:
GeneTex
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 ganglioside 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 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II). Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2014]
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