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

Name: Human Recombinant HEXA (Subunit A) protein, His Tag
SKU: V2LY-0526-LY4542
Rating: 5 (1 reviews)

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Datasheet

SDS

Request for COA

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

Basic Information

Expressed Host

Baculovirus-Insect Cells

Protein Species

Human

Tag

His Tag

Protein Construction

This product is Human Recombinant HEXA (Subunit A) protein, His Tag consist of Amino Acid: 1-529 and predicts a molecular mass of 59.7 kDa.

Molecule Mass

59.7 kDa

Sequence

Amino Acid: 1-529

Species

Human

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

Purity

>95% as determined by SDS-PAGE

Endotoxin

Please contact us for more information.

Format

Lyophilized

Reconstitution

Allow the vial and reconstitution buffer to equilibrate to room temperature. Briefly centrifuge or tap down the vial to ensure that all lyophilized powder is collected at the bottom of the vial. For the reconstitution of this product, we recommend adding PBS or sterile water to achieve a final antibody concentration of 1 mg/mL. Allow the vial to reconstitute for 10-15 minutes at room temperature with gentle agitation. Avoid vigorous shaking that can cause foaming and antibody denaturation. Aliquot into volumes based on your experiment and store liquid protein at -20°C or -80°C for long time.

Buffer

Lyophilized from sterile PBS

Preservative

None

Storage

Samples are stable for up to twelve months from date of receipt at -20°C to -80°C. Store it under sterile conditions at -20°C to -80°C. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.

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

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

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