Search :
Sign in or Register  
Welcome Sign in or Don't have an account?Register

Mouse Anti-HEXb Recombinant Antibody (CBFYH-0979) (CBMAB-H1918-FY)


See all HEXb antibodies
Online Inquiry

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBFYH-0979
Application
WB, ICC, IHC-P, IHC-Fr, ELISA

Basic Information

Specificity
Human
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

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

Format
Liquid
Buffer
PBS, pH 7.4, 50% glycerol
Preservative
0.02% Sodium azide
Concentration
0.5 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Hexosaminidase Subunit Beta
Introduction
Hexosaminidase B is the beta subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II). Alternatively spliced transcript variants encoding different isoforms have been found for this gene.
Entrez Gene ID
3074
UniProt ID
P07686
Alternative Names
Hexosaminidase Subunit Beta; N-Acetyl-Beta-Glucosaminidase Subunit Beta; Beta-N-Acetylhexosaminidase Subunit Beta; Cervical Cancer Proto-Oncogene 7 Protein; Hexosaminidase B (Beta Polypeptide); Beta-Hexosaminidase Subunit Beta; Hexosaminidase Subunit B; EC 3.2.1.5
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.
More Infomation

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.

Ask a question We look forward to hearing from you.
0 reviews or Q&As

Purchasing FAQs
Technical FAQs

Loading...
Have you used Mouse Anti-HEXb Recombinant Antibody (CBFYH-0979)?
Submit a review and get a Coupon or an Amazon gift card. 20% off Coupon $30 eGift Card
Submit a review
Loading...
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.).

Learn more

Documents

Request bulk or custom quote Online Inquiry
Second antibody and isotype control

Contact us

  • Tel: (USA)
  • (UK)
  • Fax:
  • Email:

Submit A Review

Online Inquiry