Mouse Recombinant HEXB protein, His Tag (V2LY-0526-LY8397)

Name: Mouse Recombinant HEXB protein, His Tag
SKU: V2LY-0526-LY8397
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

HEK293 Cells

Protein Species

Mouse

Tag

His Tag

Protein Construction

This product is Mouse Recombinant HEXB protein, His Tag consist of Amino Acid: 1-536 and predicts a molecular mass of 59.4 kDa.

Molecule Mass

59.4 kDa

Sequence

Amino Acid: 1-536

Species

Mouse

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 Beta

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 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).

During fertilization is responsible, at least in part, for the zona block to polyspermy. Present in the cortical granules of non-activated oocytes, is exocytosed during the cortical reaction in response to oocyte activation and inactivates the sperm galactosyltransferase-binding site, accounting for the block in sperm binding to the zona pellucida (By similarity).

Biological Process

Astrocyte cell migration Source: Ensembl
Cellular calcium ion homeostasis Source: Ensembl
Cellular protein metabolic process Source: Ensembl
Ganglioside catabolic process Source: UniProtKB
Glycosaminoglycan metabolic process Source: ComplexPortal
Lipid storage Source: Ensembl
Locomotory behavior Source: Ensembl
Lysosome organization Source: Ensembl
Male courtship behavior Source: Ensembl
Myelination Source: Ensembl
Neuromuscular process controlling balance Source: Ensembl
Oligosaccharide catabolic process Source: Ensembl
Oogenesis Source: Ensembl
Penetration of zona pellucida Source: Ensembl
Phospholipid biosynthetic process Source: Ensembl
Positive regulation of transcription by RNA polymerase II Source: Ensembl
Regulation of cell shape Source: Ensembl
Sensory perception of sound Source: Ensembl
Single fertilization Source: UniProtKB
Skeletal system development Source: Ensembl

Cellular Location

Lysosome; Cortical granule

Involvement in disease

GM2-gangliosidosis 2 (GM2G2):
An autosomal recessive lysosomal storage disease marked by the accumulation of GM2 gangliosides in the neuronal cells. Clinically indistinguishable from GM2-gangliosidosis type 1, presenting startle reactions, early blindness, progressive motor and mental deterioration, macrocephaly and cherry-red spots on the macula.

PTM

N-linked glycans at Asn-142 and Asn-190 consist of Man3-GlcNAc2 and Man(5 to 7)-GlcNAc2, respectively.
The beta-A and beta-B chains are produced by proteolytic processing of the precursor beta chain.

Xie, H., Lin, S., Chen, Y., Wang, W., Qi, Y., Li, J., ... & Feng, X. (2023). A case of Sandhoff disease caused by a novel β-hexosaminidase B (HEXB) mutation c. 118delG (p. A40fs* 24): A case report from China. Medicine, 102(24).

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.

Whyte, L. S., Fourrier, C., Hassiotis, S., Lau, A. A., Trim, P. J., Hein, L. K., ... & Sargeant, T. J. (2022). Lysosomal gene Hexb displays haploinsufficiency in a knock-in mouse model of Alzheimer’s disease. IBRO Neuroscience Reports, 12, 131-141.

Rahmani, Z., Banisadr, A., Ghodsinezhad, V., Dibaj, M., & Aryani, O. (2022). P. Ala278Val mutation might cause a pathogenic defect in HEXB folding leading to the Sandhoff disease. Metabolic Brain Disease, 37(8), 2669-2675.

Masuda, T., Amann, L., Sankowski, R., Staszewski, O., Lenz, M., d´ Errico, P., ... & Prinz, M. (2020). Novel Hexb-based tools for studying microglia in the CNS. Nature immunology, 21(7), 802-815.

Ornaghi, F., Sala, D., Tedeschi, F., Maffia, M. C., Bazzucchi, M., Morena, F., ... & Gritti, A. (2020). Novel bicistronic lentiviral vectors correct β-Hexosaminidase deficiency in neural and hematopoietic stem cells and progeny: implications for in vivo and ex vivo gene therapy of GM2 gangliosidosis. Neurobiology of disease, 134, 104667.

Sung, A. R., Moretti, P., & Shaibani, A. (2018). Case of late-onset Sandhoff disease due to a novel mutation in the HEXB gene. Neurology Genetics, 4(4).

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.

Li, X., An, Y., Liao, J., Xiao, L., Swanson, M., Martinez‐Fonts, K., ... & Richardson, D. D. (2021). Identification and characterization of a residual host cell protein hexosaminidase B associated with N‐glycan degradation during the stability study of a therapeutic recombinant monoclonal antibody product. Biotechnology progress, 37(3), e3128.

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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
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Alternative Versions

Human Recombinant HEXB protein, His Tag (CAT#: V2LY-0526-LY4543)

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Isotype control

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

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