Human HEXA ELISA Kit (V2LY-0626-LY5518)

Name: Human HEXA ELISA Kit
SKU: V2LY-0626-LY5518
Rating: 5 (1 reviews)

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Tested Data

Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Sensitivity

0.0049 ng/mL

Detection Range

0.01-2 ng/mL

Sample Type

Serum, Plasma, cell culture supernates

Specificity

Human

Assay Type

Sandwich

Reactivity

Human

Assay Time

1.5 h

Molecule Mass

60.7 kDa

Components

Pre-coated ELISA plate: 12 wells * 8 detachable strips
Standard solution: 0.5ml x1
Standard diluent: 3ml x1
Streptavidin-HRP: 6ml x1
Stop solution: 6ml x1
Substrate solution A: 6ml x1
Substrate solution B: 6ml x1
Wash buffer concentrate (25x): 20ml x1
Biotinylated antibody: 1ml x1

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

Storage

Store at 2-8°C

More Infomation

Target

Full Name

Hexosaminidase Subunit Alpha

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).

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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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)
Proteogenomic
Other Protocols

Alternative Versions

Human Recombinant HEXA (Subunit A) protein, His Tag (CAT#: V2LY-0526-LY4542)

Rat Hexa ELISA Kit (CAT#: V2LY-0626-LY2091)

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Rat Hexa ELISA Kit (CAT#: V2LY-0626-LY2091)

Human Recombinant HEXA (Subunit A) protein, His Tag (CAT#: V2LY-0526-LY4542)

Mouse Anti-HEXA Recombinant Antibody (A160) (CAT#: CBMAB-AP9589LY)

Isotype control

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

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