Mouse Anti-HEXA Recombinant Antibody (A160) (CBMAB-AP9589LY)

Name: Mouse Anti-HEXA Recombinant Antibody (A160)
SKU: CBMAB-AP9589LY
Rating: 5 (1 reviews)

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Datasheet

SDS

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

Basic Information

Host Animal

Mouse

Clone

A160

Application

ELISA, WB

Immunogen

Fusion protein of HEXA

Specificity

Human

Antibody Isotype

IgG2b

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

Purity

Affinity purity

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

More Infomation

Target

Full Name

Hexosaminidase Subunit Alpha

Introduction

This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I). Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed. [provided by RefSeq, Jan 2016]

Entrez Gene ID

3073

UniProt ID

P06865

Alternative Names

Hexosaminidase Subunit Alpha; N-Acetyl-Beta-Glucosaminidase Subunit Alpha; Beta-N-Acetylhexosaminidase Subunit Alpha; Hexosaminidase A (Alpha Polypeptide); Beta-Hexosaminidase Subunit Alpha; Hexosaminidase Subunit A;

Function

E-AB-27475

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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Isotype control

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.).

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