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

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

Host Animal

Mouse

Clone

CBFYH-0979

Application

WB, ICC, IHC-P, IHC-Fr, ELISA

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.

More Infomation

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.

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)
Proteogenomics
Other Protocols

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

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