HEXA antibody
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Name
HEXA antibody
Category
Primary Antibodies
Provider
FineTest
Reference
FNab03843
Tested Applications
ELISA, WB
Description
Responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. The form B is active against certain oligosaccharides. The form S has no measurable activity.
Documentos del producto
Especificaciones del producto
| Category | Primary Antibodies |
| Immunogen Target | hexosaminidase A(alpha polypeptide) (HEXA) |
| Host | Mouse |
| Reactivity | Human |
| Recommended Dilution | WB: 1:5000 - 1:20000 |
| Clonality | monoclonal |
| Conjugation | Unconjugated |
| Isotype | IgG2a |
| Clone ID | 4F7 |
| Observed MW | 60 kDa |
| Purity | ≥95% as determined by SDS-PAGE |
| Purification | Protein A+G purification |
| Size 1 | 100µg |
| Form | liquid |
| Tested Applications | ELISA, WB |
| Storage | PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20℃ for 12 months(Avoid repeated freeze / thaw cycles.) |
| UniProt ID | P06865 |
| Gene ID | 3073 |
| Alias | N-acetyl-beta-glucosaminidase subunit alpha,TSD |
| Background | Antibody anti-HEXA |
| Status | RUO |
| Note | Mol. Weight 60 kDa |
Background
HEXA is the alpha subunit of the beta-hexosaminidase enzyme, a lysosomal hydrolase that catalyzes the breakdown of GM2 gangliosides into GM3 in the process of glycosphingolipid degradation The enzyme functions as a heterodimer composed of alpha and beta subunits, encoded by the HEXA and HEXB genes respectively Mutations in HEXA lead to Tay-Sachs disease, an autosomal recessive lysosomal storage disorder characterized by the accumulation of GM2 gangliosides in neuronal cells, resulting in neurodegeneration, progressive motor weakness, and developmental delay HEXA is highly active in neuronal tissues where glycosphingolipid metabolism is critical for normal brain function The absence or dysfunction of HEXA causes a loss of enzymatic activity leading to cellular toxicity and apoptosis Current therapeutic approaches include enzyme replacement therapy, substrate reduction therapy, and gene therapy to restore HEXA activity and slow disease progression
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