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Beta氨基己糖苷酶beta亚基蛋白B链抗体
  • 产品货号：
    BN40458R
  • 中文名称：
    Beta氨基己糖苷酶beta亚基蛋白B链抗体
  • 英文名称：
    Rabbit anti-HEXB chain B Polyclonal antibody

  • 货号

    产品规格

    售价

    备注

  • BN40458R-100ul

    100ul

    ¥2360.00

    交叉反应：nan(predicted:Human,Mouse,Rat,Dog,Cow,Sheep) 推荐应用：WB,IHC-P,IHC-F,ICC,IF,ELISA

  • BN40458R-200ul

    200ul

    ¥3490.00

    交叉反应：nan(predicted:Human,Mouse,Rat,Dog,Cow,Sheep) 推荐应用：WB,IHC-P,IHC-F,ICC,IF,ELISA

英文名称HEXB chain B
中文名称Beta氨基己糖苷酶beta亚基蛋白B链抗体
别    名Beta hexosaminidase beta chain; Beta hexosaminidase subunit beta; Beta N acetylhexosaminidase; Beta-hexosaminidase subunit beta chain B; Beta-N-acetylhexosaminidase subunit beta; Cervical cancer proto oncogene 7 protein; Cervical cancer proto-oncogene 7 protein; ENC 1AS; HCC 7; HCC-7; HCC7; HEX B; Hexb; HEXB_HUMAN; Hexosaminidase B (beta polypeptide); Hexosaminidase B; Hexosaminidase subunit B; HexosaminidaseB; N acetyl beta glucosaminidase; N-acetyl-beta-glucosaminidase subunit beta; HEXB chain B.   
研究领域细胞生物  免疫学  信号转导  新陈代谢  
抗体来源Rabbit
克隆类型Polyclonal
交叉反应(predicted: Human, Mouse, Rat, Dog, Cow, Sheep, )
产品应用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量22/50kDa
细胞定位细胞浆 
性    状Liquid
浓    度1mg/ml
免 疫 原KLH conjugated synthetic peptide derived from human HEXB:201-300/556 
亚    型IgG
纯化方法affinity purified by Protein A
储 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
产品介绍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). [provided by RefSeq, Jul 2008].

Function:
Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues.

Subunit:
There are 3 forms of beta-hexosaminidase: hexosaminidase A is a trimer composed of one subunit alpha, one subunit beta chain A and one subunit beta chain B; hexosaminidase B is a tetramer of two subunit beta chains A and two subunit beta chains B; hexosaminidase S is a homodimer of two alpha subunits. The two beta chains are derived from the cleavage of the beta subunit.

Subcellular Location:
Lysosome.

DISEASE:
GM2-gangliosidosis 2 (GM2G2) [MIM:268800]: An autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. Clinically indistinguishable from GM2-gangliosidosis type 1, presenting startle reactions, early blindness, progressive motor and mental deterioration, macrocephaly and cherry-red spots on the macula. Note=The disease is caused by mutations affecting the gene represented in this entry.

Similarity:
Belongs to the glycosyl hydrolase 20 family.

SWISS:
P07686

Gene ID:
3074

Database links:

Entrez Gene: 3074 Human

GenBank: NP_000512.1 Human

Omim: 606873 Human

SwissProt: P07686 Human

Unigene: 69293 Human



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.



















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