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anti-HEXA antibody :: Rabbit HEXA Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS9127446
Western Blot (WB)
Unit / Price
0.05 mL  /  $230 +1 FREE 8GB USB
0.1 mL  /  $305 +1 FREE 8GB USB
0.2 mL  /  $460 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

HEXA, Polyclonal Antibody

★Popular Item★
 Also Known As   

HEXA Polyclonal Antibody

 Product Synonym Names    TSD
 Product Gene Name   

anti-HEXA antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    phenotype 606869
 3D Structure    ModBase 3D Structure for P06865
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human, Mouse, Rat
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 Purity/Purification    Affinity Purification
 Concentration    1mg/ml (lot specific)
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 Species    Human
 Immunogen    Recombinant Protein
 Immunogen    Recombinant protein of human HEXA
 Calculated Molecular Weight    60kDa
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 Preparation and Storage    Store at -20 degree C (regular) or -80 degree C (long term). Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
 Other Notes    Small volumes of anti-HEXA antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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Related Product Information for anti-HEXA antibody

   This gene encodes the alpha 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. Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I).
 Product Categories/Family for anti-HEXA antibody    Polyclonal
 Applications Tested/Suitable for anti-HEXA antibody   

Western Blot (WB), Immunohistochemistry (IHC)

 Application Notes for anti-HEXA antibody    WB: 1:500 - 1:2000
IHC: 1:50 - 1:200
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 Western Blot (WB) of anti-HEXA antibody    Western blot analysis of extracts of various cell lines, using HEXA antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 5s.
anti-HEXA antibody Western Blot (WB) (WB) image
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NCBI/Uniprot data below describe general gene information for HEXA. It may not necessarily be applicable to this product.
 NCBI GI #    311033393
 NCBI GeneID    3073
 NCBI Accession #    P06865.2 [Other Products]
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 UniProt Primary Accession #    P06865 [Other Products]
 UniProt Secondary Accession #    Q53HS8; Q6AI32; B4DKE7; E7ENH7 [Other Products]
 UniProt Related Accession #    P06865 [Other Products]
 Molecular Weight    529
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 NCBI Official Full Name    Beta-hexosaminidase subunit alpha
 NCBI Official Synonym Full Names    hexosaminidase A (alpha polypeptide)
 NCBI Official Symbol    HEXA [Similar Products]
 NCBI Official Synonym Symbols   
TSD
[Similar Products]
 NCBI Protein Information    beta-hexosaminidase subunit alpha; hexosaminidase subunit A; beta-N-acetylhexosaminidase subunit alpha; N-acetyl-beta-glucosaminidase subunit alpha
 UniProt Protein Name    Beta-hexosaminidase subunit alpha
 UniProt Synonym Protein Names   
Beta-N-acetylhexosaminidase subunit alpha; Hexosaminidase subunit A; N-acetyl-beta-glucosaminidase subunit alpha
 Protein Family    Hexamerin
 UniProt Gene Name    HEXA [Similar Products]
 UniProt Synonym Gene Names    Hexosaminidase subunit A [Similar Products]
 UniProt Entry Name    HEXA_HUMAN
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 NCBI Summary for HEXA    This gene encodes the alpha 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. Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I). [provided by RefSeq, Jul 2009]
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 UniProt Comments for HEXA    HEXA: 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. Defects in HEXA are the cause of GM2-gangliosidosis type 1 (GM2G1); 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). Belongs to the glycosyl hydrolase 20 family.

Protein type: Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - glycosaminoglycan degradation; Carbohydrate Metabolism - amino sugar and nucleotide sugar; Hydrolase; Glycan Metabolism - other glycan degradation; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; EC 3.2.1.52

Chromosomal Location of Human Ortholog: 15q24.1

Cellular Component: lysosomal lumen; membrane

Molecular Function: protein heterodimerization activity; beta-N-acetylhexosaminidase activity

Biological Process: keratan sulfate metabolic process; chondroitin sulfate metabolic process; sphingolipid metabolic process; glycosaminoglycan metabolic process; chondroitin sulfate catabolic process; carbohydrate metabolic process; glycosphingolipid metabolic process; pathogenesis; keratan sulfate catabolic process; hyaluronan metabolic process; hyaluronan catabolic process

Disease: Tay-sachs Disease
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 Research Articles on HEXA    1. Human prostate cancer cells are characterised by a significant decrease in HexA activity.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-HEXA antibodyPathways associated with anti-HEXA antibody
 Reference Product  PubMed Publications
 HEXB antibody  >79 publications with HEXA and HEXB
 GM2A antibody  >9 publications with HEXA and GM2A
 NAGA antibody  >4 publications with HEXA and NAGA
 GLA antibody  >3 publications with HEXA and GLA
 GUSB antibody  >3 publications with HEXA and GUSB
 IDS antibody  >2 publications with HEXA and IDS
 GLB1 antibody  >2 publications with HEXA and GLB1
 Products by Pathway  Pathway Diagram
 Amino Sugar And Nucleotide Sugar Metabolism Pathway antibodies  Amino Sugar And Nucleotide Sugar Metabolism Pathway Diagram
 Amino Sugar And Nucleotide Sugar Metabolism Pathway antibodies  Amino Sugar And Nucleotide Sugar Metabolism Pathway Diagram
 CS/DS Degradation Pathway antibodies  CS/DS Degradation Pathway Diagram
 Chondroitin Sulfate/dermatan Sulfate Metabolism Pathway antibodies  Chondroitin Sulfate/dermatan Sulfate Metabolism Pathway Diagram
 Defective B3GAT3 Causes JDSSDHD Pathway antibodies  Defective B3GAT3 Causes JDSSDHD Pathway Diagram
 Defective B4GALT1 Causes B4GALT1-CDG (CDG-2d) Pathway antibodies  Defective B4GALT1 Causes B4GALT1-CDG (CDG-2d) Pathway Diagram
 Defective B4GALT7 Causes EDS, Progeroid Type Pathway antibodies  Defective B4GALT7 Causes EDS, Progeroid Type Pathway Diagram
 Defective CHST14 Causes EDS, Musculocontractural Type Pathway antibodies  Defective CHST14 Causes EDS, Musculocontractural Type Pathway Diagram
 Defective CHST3 Causes SEDCJD Pathway antibodies  Defective CHST3 Causes SEDCJD Pathway Diagram
 Defective CHST6 Causes MCDC1 Pathway antibodies  Defective CHST6 Causes MCDC1 Pathway Diagram
Diseases associated with anti-HEXA antibodyOrgans/Tissues associated with anti-HEXA antibody
 Disease Name  Pubmed Publications
 Tay-Sachs Disease Antibodies  >133 publications with HEXA and Tay-Sachs Disease
 Hepatitis Antibodies  >62 publications with HEXA and Hepatitis
 Liver Diseases Antibodies  >39 publications with HEXA and Liver Diseases
 Inflammation Antibodies  >36 publications with HEXA and Inflammation
 Carcinoma Antibodies  >33 publications with HEXA and Carcinoma
 Death Antibodies  >29 publications with HEXA and Death
 Necrosis Antibodies  >28 publications with HEXA and Necrosis
 Poisoning Antibodies  >22 publications with HEXA and Poisoning
 Cardiovascular Diseases Antibodies  >21 publications with HEXA and Cardiovascular Diseases
 Kidney Diseases Antibodies  >20 publications with HEXA and Kidney Diseases
 Organ/Tissue Name  Pubmed Publications
 Blood Antibodies  >319 publications with HEXA and Blood
 Liver Antibodies  >157 publications with HEXA and Liver
 Brain Antibodies  >119 publications with HEXA and Brain
 Muscle Antibodies  >89 publications with HEXA and Muscle
 Kidney Antibodies  >70 publications with HEXA and Kidney
 Connective Tissue Antibodies  >42 publications with HEXA and Connective Tissue
 Lung Antibodies  >41 publications with HEXA and Lung
 Intestine Antibodies  >40 publications with HEXA and Intestine
 Skin Antibodies  >40 publications with HEXA and Skin
 Heart Antibodies  >37 publications with HEXA and Heart
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