Mouse Anti-HEXA Recombinant Antibody (CBT3891) (V2LY-0625-LY2686)

Name: Mouse Anti-HEXA Recombinant Antibody (CBT3891)
SKU: V2LY-0625-LY2686
Rating: 5 (1 reviews)

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Datasheet

Summary

Host Animal

Mouse

Specificity

Human

Clone

CBT3891

Antibody Isotype

IgG2b

Application

WB, FC

Basic Information

Immunogen

Purified recombinant fragment of human HEXA expressed in E. Coli.

Host Species

Mouse

Specificity

Human

Antibody Isotype

IgG2b

Clonality

Monoclonal Antibody

Application Notes

Application	Note
WB	1:500-1:2,000
FC	1:200-1:400
ELISA	1:10,000

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Buffer

PBS

Preservative

Sodium azide

Concentration

Batch dependent

Purity

> 95% Purity determined by SDS-PAGE.

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name

Hexosaminidase Subunit Alpha

Entrez Gene ID

3073

UniProt ID

P06865

Function

Hydrolyzes the non-reducing end N-acetyl-D-hexosamine and/or sulfated N-acetyl-D-hexosamine of glycoconjugates, such as the oligosaccharide moieties from proteins and neutral glycolipids, or from certain mucopolysaccharides (PubMed:11707436, PubMed:9694901, PubMed:8672428, PubMed:8123671).

The isozyme S is as active as the isozyme A on the anionic bis-sulfated glycans, the chondroitin-6-sulfate trisaccharide (C6S-3), and the dermatan sulfate pentasaccharide, and the sulfated glycosphingolipid SM2 (PubMed:11707436).

The isozyme B does not hydrolyze each of these substrates, however hydrolyzes efficiently neutral oligosaccharide (PubMed:11707436).

Only the isozyme A is responsible for the degradation of GM2 gangliosides in the presence of GM2A (PubMed:9694901, PubMed:8672428, PubMed:8123671).

Biological Process

Carbohydrate metabolic process Source: InterPro
Ganglioside catabolic process Source: UniProtKB
Glycosaminoglycan biosynthetic process Source: UniProtKB
Glycosaminoglycan metabolic process Source: ComplexPortal

Cellular Location

Lysosome

Involvement in disease

GM2-gangliosidosis 1 (GM2G1):
An autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. It is characterized by GM2 gangliosides accumulation in the absence of HEXA activity, leading to neurodegeneration and, in the infantile form, death in early childhood. It exists in several forms: infantile (most common and most severe), juvenile and adult (late-onset).

PTM

N-linked glycan at Asn-115 consists of Man3-GlcNAc2 (PubMed:1533633, PubMed:16698036, PubMed:19159218) (Probable). N-linked glycan at Asn-157 consists of either GlcNAc or GlcNAc2-Man7-9. N-linked glycan at Asn-295 consists of either GlcNAc, GlcNAc-Fuc, or GlcNAc2-Man4 (Probable).

More Infomation

References

Shaimardanova, A. A., Chulpanova, D. S., Solovyeva, V. V., Issa, S. S., Mullagulova, A. I., Titova, A. A., ... & Rizvanov, A. A. (2024). Increasing β-hexosaminidase A activity using genetically modified mesenchymal stem cells. Neural Regeneration Research, 19(1), 212-219.

Shaimardanova, A. A., Chulpanova, D. S., Solovyeva, V. V., Aimaletdinov, A. M., & Rizvanov, A. A. (2022). Functionality of a bicistronic construction containing HEXA and HEXB genes encoding β-hexosaminidase A for cell-mediated therapy of GM2 gangliosidoses. Neural Regeneration Research, 17(1), 122.

Bertani, V., Prioni, S., Di Lecce, R., Gazza, F., Ragionieri, L., Merialdi, G., ... & Cantoni, A. M. (2021). A pathogenic HEXA missense variant in wild boars with Tay-Sachs disease. Molecular genetics and metabolism, 133(3), 297-306.

Montgomery, M. K., Taddese, A. Z., Bayliss, J., Nie, S., Williamson, N. A., & Watt, M. J. (2021). Hexosaminidase A (HEXA) regulates hepatic sphingolipid and lipoprotein metabolism in mice. The FASEB Journal, 35(12), e22046.

Shaimardanova, A. A., Chulpanova, D. S., Solovyeva, V. V., Garanina, E. E., Salafutdinov, I. I., Laikov, A. V., ... & Rizvanov, A. A. (2021). Serum cytokine profile, beta-hexosaminidase a enzymatic activity and gm2 ganglioside levels in the plasma of a tay-sachs disease patient after cord blood cell transplantation and curcumin administration: A case report. Life, 11(10), 1007.

Toro, C., Shirvan, L., & Tifft, C. (2020). HEXA disorders.

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Protocols

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

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Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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SDS
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Second antibody and isotype control

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