Anti-HEXB chain B抗体-抗体-抗体-生物在线

Anti-HEXB chain B抗体

商家询价

产品名称： Anti-HEXB chain B抗体

英文名称： HEXB chain B

产品编号： YB--15468R

产品价格： null

产品产地：中国/美国

品牌商标： Ybscience

更新时间： 2023-08-17T10:29:50

使用范围：科研使用

上海钰博生物科技有限公司

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产品详情

Anti-HEXB chain B抗体

产品编号	YB-15468R
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.
规格价格	100ul/1580元购买 200ul/2480元购买大包装/询价
说明书	100ul 200ul
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	Human, Mouse, Rat, Dog, Cow, Sheep,
产品应用	WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 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	Lyophilized or 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	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
PubMed	PubMed
产品介绍	background: 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.