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Mouse Anti-HEXA Recombinant Antibody (CBYJT-1363) (CBMAB-T0385-YJ)

Provided herein is a Mouse monoclonal antibody, which binds to HEXA (Hexosaminidase Subunit Alpha). The antibody can be used for immunoassay techniques, such as WB, ICC, IHC-P, IHC-Fr, ELISA.
See all HEXA antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBYJT-1363
Antibody Isotype
IgG
Application
WB, ICC, IHC-P, IHC-Fr, ELISA

Basic Information

Immunogen
Hexosaminidase A Alpha
Specificity
Human
Antibody Isotype
IgG
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!]

Buffer
PBS, pH 7.4, 50% Glycerol
Preservative
0.02% Sodium Azide
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 Alpha
Introduction
HEXA is 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. HEXA, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in HEXA 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).
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
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).
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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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