Western Blot (WB)
(Anti- HEXA antibody, MBS176966, Western blottingAll lanes: Anti HEXA (MBS176966) at 0.5ug/mlLane 1: Human Placenta Tissue Lysate at 50ugLane 2: HELA Whole Cell Lysate at 40ugLane 3: HEPG2 Whole Cell Lysate at 40ugLane 4: U87 Whole Cell Lysate at 40ugPredicted bind size: 61KDObserved bind size: 61KD )
Rabbit anti-Human HEXA Polyclonal Antibody | anti-HEXA antibody
Anti-HEXA antibody
Western Blot (WB)
(Anti- HEXA antibody, MBS176966, Western blottingAll lanes: Anti HEXA (MBS176966) at 0.5ug/mlLane 1: Human Placenta Tissue Lysate at 50ugLane 2: HELA Whole Cell Lysate at 40ugLane 3: HEPG2 Whole Cell Lysate at 40ugLane 4: U87 Whole Cell Lysate at 40ugPredicted bind size: 61KDObserved bind size: 61KD )
Western Blot (WB)
(Anti- HEXA antibody, MBS176966, Western blottingAll lanes: Anti HEXA (MBS176966) at 0.5ug/mlLane 1: Human Placenta Tissue Lysate at 50ugLane 2: HELA Whole Cell Lysate at 40ugLane 3: HEPG2 Whole Cell Lysate at 40ugLane 4: U87 Whole Cell Lysate at 40ugPredicted bind size: 61KDObserved bind size: 61KD )
Background: HEXA(hexosaminidase A(alpha polypeptide)) is an enzyme that in humans is encoded by the HEXA gene. Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines The HEXA gene encodes the alpha subunit of hexosaminidase A, a lysosomal enzyme involved in the breakdown of gangliosides. The HEXA gene is mapped on 15q23. Even though the alpha and beta subunits of hexosaminidase A can both cleave GalNAc residues, only the alpha subunit is able to hydrolyze GM2 gangliosides. The alpha subunit contains a key residue, Arg-424, which is essential for binding the N-acetyl-neuramanic residue of GM2 gangliosides. Chimeric constructs were expressed in HeLa cells and selected constructs were produced in the baculovirus expression system to determine their ability to degrade GM2 ganglioside in the presence of GM2 activator protein. Their results allowed them to define 2 noncontiguous sequences in the alpha subunit(amino acids 1-191 and 403-529) which, when substituted into analogous positions in the beta subunit, conferred activity against the sulfated substrate.
NCBI and Uniprot Product Information
NCBI Description
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 Description
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 - globo series; Hydrolase; Carbohydrate Metabolism - amino sugar and nucleotide sugar; Glycan Metabolism - glycosaminoglycan degradation; EC 3.2.1.52; Glycan Metabolism - glycosphingolipid biosynthesis - ganglio series; Glycan Metabolism - other glycan degradation
Chromosomal Location of Human Ortholog: 15q24.1
Cellular Component: lysosomal lumen; membrane
Molecular Function: protein heterodimerization activity; beta-N-acetylhexosaminidase activity
Biological Process: chondroitin sulfate metabolic process; keratan 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
Research Articles on HEXA
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Product Notes
The HEXA hexa (Catalog #AAA176966) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The Anti-HEXA antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's HEXA can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Researchers should empirically determine the suitability of the HEXA hexa for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "HEXA, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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