Anti-HEXB Products

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For Research Use Only. Not For Clinical Use.

Creative Biolabs is a leading provider of research tools for rare disease. Our proprietary anti-HEXB recombinant antibodies are designed for high specificity to meet your needs in developing and evaluating therapeutics for the debilitating disease known as Sandhoff disease.

HEXB: The Common Subunit Deficient in Sandhoff Disease

Hexosaminidase Subunit Beta (HEXB) is a subunit common to two critical lysosomal enzymes. HEXB can homodimerize to form Hexosaminidase B (Hex B), or heterodimerize with Hexosaminidase Subunit Alpha (HEXA) to form Hexosaminidase A (Hex A). Hex A and Hex B have important and overlapping functions in glycosphingolipid degradation. Hex A, for example, is well known for its ability to degrade GM2 ganglioside. A mutation in the HEXB gene disrupts the production of both Hex A and Hex B enzymes. This results in an increased and simultaneous lysosomal accumulation of both GM2 ganglioside and several other Hex A and Hex B substrates. The massive lysosomal storage that occurs causes Sandhoff disease, a severe and progressive neurodegenerative disorder, which is also one form of GM2 gangliosidosis.

Alternative Names

ENC-1AS; HEL-248; HEL-S-111

Background

Hexosaminidase B (beta polypeptide) is the beta subunit of lysosomal enzyme beta-hexosaminidase. The enzyme, together with cofactor GM2 activator protein, hydrolyzes the ganglioside GM2 and other terminal N-acetyl hexosamines. Beta-hexosaminidase has two subunits, alpha and beta, which are encoded by separate genes. Beta-hexosaminidase alpha and beta subunits are both in family 20 of the glycosyl hydrolases. Mutations in either the alpha or the beta subunit gene lead to a buildup of GM2 ganglioside in neurons and to the neurodegenerative disorders known as GM2 gangliosidoses. Mutations in the beta subunit gene specifically lead to Sandhoff disease (GM2-gangliosidosis type II). Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

Anti-HEXB rAb Products

Creative Biolabs anti-HEXB recombinant antibodies are highly specific and sensitive, and fully validated for Western Blot to use in patient cells to verify the absence of the beta subunit and IHC to verify distribution of the protein product in gene therapy approaches. These rAbs are highly reliable for diagnostics and research in Sandhoff disease.

Table 1. Featured anti-HEXB recombinant antibody products at Creative Biolabs.

Customer Reviews

rAb Production

Creative Biolabs has access to the most advanced antibody platforms to bring rapid advancements in research for these catastrophic neurological disorders to fruition. Creative Biolabs anti-HEXB recombinant antibodies are expressed in the best possible system for this protein to ensure your antibody will be able to specifically recognize native protein with a high degree of biological relevance and activity for your research.

Featured Anti-HEXB Recombinant Antibody Production Platforms
Fig.1 Milligram-scale anti-HEXB recombinant antibody production.

Fig.2 Gram-scale anti-HEXB recombinant antibody production.

rAb Modalities

Creative Biolabs is passionate about providing the neuro-genetics community with the most adaptable tools possible. Our anti-HEXB recombinant antibodies can be produced in a wide array of formats to customize for your experimental needs so that the research community around the world can be developing and testing new therapeutic approaches for Sandhoff disease.

Fig.3 Full-length anti-HEXB recombinant antibody production and modalities.

Power your research to fight Sandhoff disease with Creative Biolabs' high specificity anti-HEXB recombinant antibodies. All of the products in this portfolio are some of the most thoroughly validated in the industry so you can have confidence that you're getting exactly what you need for your experiments. Don't leave your research to chance. Let us help. Contact one of our specialists today.