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HEXA Polyclonal Antibody

商品貨號： PLA018941

適應(yīng) 性： 人,小鼠,大鼠

￥600元

規(guī)格：

40μL 100μL 200μL

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MSDS

說明書

商品描述

基因名稱： HEXA
蛋白名稱： Beta-hexosaminidase subunit alpha
Human_gene_id： 3073
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=3073
Human_swiss_prot_no： P06865
Human_swiss_link： http://www.uniprot.org/uniprotkb/P06865/entry
Mouse_gene_id： 15211
Mouse_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=15211
Mouse_swiss_prot_no： P29416
Mouse_swiss_link： http://www.uniprot.org/uniprot/P29416
Rat_gene_id： 300757
Rat_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=300757
Rat_swiss_prot_no： Q641X3
Rat_swiss_link： http://www.uniprot.org/uniprot/Q641X3
特異性： HEXA Polyclonal Antibody detects endogenous levels of HEXA protein.
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來源： Polyclonal, Rabbit,IgG
稀釋： WB 1:500 - 1:2000. IHC-p: 1:100-300 ELISA: 1:20000.. IF 1:50-200
純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度： 1 mg/ml
儲存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
其他名稱： HEXA; Beta-hexosaminidase subunit alpha; Beta-N-acetylhexosaminidase subunit alpha; Hexosaminidase subunit A; N-acetyl-beta-glucosaminidase subunit alpha
實測條帶： 60kD
信號通路： Other glycan degradation;Amino sugar and nucleotide sugar metabolism;Glycosaminoglycan degradation;Glycosphingolipid biosynthesis;Glycosphingolipid biosynthesis;Lysosome;
功能： catalytic activity:Hydrolysis of terminal non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides.,disease:Defects in HEXA are the cause of GM2-gangliosidosis type 1 (GM2G1) [MIM:272800]; also known as Tay-Sachs disease. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G1 is characterized by GM2 gangliosides accumulation in the absence of HEXA activity, leading to neurodegeneration and, in the infantile form, death in early childhood. GM2G1 has an increased incidence among Ashkenazi Jews and French Canadians in eastern Quebec. It exists in several forms: infantile (most common and most severe), juvenile and adult (late onset).,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. The form B is active against certain oligosaccharides. The form S has no measurable activity.,online information:HEXA mutation database,online information:Tay Sachs disease website,PTM:N-linked glycan at Asn-115 consists of Man(3)-GlcNAc(2).,similarity:Belongs to the glycosyl hydrolase 20 family.,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 an homodimer of two alpha subunits. The two beta chains are derived from the cleavage of the beta subunit.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： Lysosome.
組織表達： Brain,Eye,Liver,Ovary,Uterus,
科研貨號： PLA018941

HEXA Polyclonal Antibody

Catalog No PLA018941

Product information

基因名稱： HEXA
蛋白名稱： Beta-hexosaminidase subunit alpha
Human_gene_id： 3073
Human_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=3073
Human_swiss_prot_no： P06865
Human_swiss_link： http://www.uniprot.org/uniprotkb/P06865/entry
Mouse_gene_id： 15211
Mouse_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=15211
Mouse_swiss_prot_no： P29416
Mouse_swiss_link： http://www.uniprot.org/uniprot/P29416
Rat_gene_id： 300757
Rat_gene_link： http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=300757
Rat_swiss_prot_no： Q641X3
Rat_swiss_link： http://www.uniprot.org/uniprot/Q641X3
特異性： HEXA Polyclonal Antibody detects endogenous levels of HEXA protein.
組成： Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
來源： Polyclonal, Rabbit,IgG
稀釋： WB 1:500 - 1:2000. IHC-p: 1:100-300 ELISA: 1:20000.. IF 1:50-200
純化工藝： The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
濃度： 1 mg/ml
儲存： -15°C to -25°C/1 year(Do not lower than -25°C)
說明書： 1
Msds： MSDS_Antibody.pdf
其他名稱： HEXA; Beta-hexosaminidase subunit alpha; Beta-N-acetylhexosaminidase subunit alpha; Hexosaminidase subunit A; N-acetyl-beta-glucosaminidase subunit alpha
實測條帶： 60kD
信號通路： Other glycan degradation;Amino sugar and nucleotide sugar metabolism;Glycosaminoglycan degradation;Glycosphingolipid biosynthesis;Glycosphingolipid biosynthesis;Lysosome;
功能： catalytic activity:Hydrolysis of terminal non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides.,disease:Defects in HEXA are the cause of GM2-gangliosidosis type 1 (GM2G1) [MIM:272800]; also known as Tay-Sachs disease. GM2-gangliosidosis is an autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. GM2G1 is characterized by GM2 gangliosides accumulation in the absence of HEXA activity, leading to neurodegeneration and, in the infantile form, death in early childhood. GM2G1 has an increased incidence among Ashkenazi Jews and French Canadians in eastern Quebec. It exists in several forms: infantile (most common and most severe), juvenile and adult (late onset).,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. The form B is active against certain oligosaccharides. The form S has no measurable activity.,online information:HEXA mutation database,online information:Tay Sachs disease website,PTM:N-linked glycan at Asn-115 consists of Man(3)-GlcNAc(2).,similarity:Belongs to the glycosyl hydrolase 20 family.,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 an homodimer of two alpha subunits. The two beta chains are derived from the cleavage of the beta subunit.,
相關(guān)產(chǎn)品： RS0001,RS0002,YM3028,YM3029
細(xì)胞定位： Lysosome.
組織表達： Brain,Eye,Liver,Ovary,Uterus,
科研貨號： PLA018941

Hunan UPT Biotechnology Co.,Ltd

Website：m.tjdqdt.com Servive hotline :4006916686

E-mail：service@uptbio.com

Address：

Room 402, Building 13, Xinggong International Industrial Park, 100 Guyuan Road, Yuelu District, Changsha City, Hunan Province, China.

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