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anti-HEXA antibody :: Rabbit HEXA Polyclonal Antibody

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Catalog # MBS9127446
Unit / Price
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  0.05 mL  /  $200 +1 FREE 8GB USB
  0.1 mL  /  $275 +1 FREE 8GB USB
  0.2 mL  /  $430 +1 FREE 8GB USB
Western Blot (WB)
Product Name

HEXA, Polyclonal Antibody

Popular Item
Full Product Name

HEXA Polyclonal Antibody

Product Synonym Names
TSD
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
OMIM
phenotype 606869
3D Structure
ModBase 3D Structure for P06865
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Affinity Purification
Concentration
1mg/ml (lot specific)
Species
Human
Immunogen
Recombinant Protein
Immunogen
Recombinant protein of human HEXA
Calculated Molecular Weight
60kDa
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.
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
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

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
NCBI/Uniprot data below describe general gene information for HEXA. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
529
NCBI Official Full Name
Beta-hexosaminidase subunit alpha
NCBI Official Synonym Full Names
hexosaminidase A (alpha polypeptide)
NCBI Official Symbol
NCBI Official Synonym Symbols
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
UniProt Gene Name
UniProt Synonym Gene Names
Hexosaminidase subunit A  [Similar Products]
UniProt Entry Name
HEXA_HUMAN
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]
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
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.

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