Beta-Hexosaminidase Subunit Alpha (HEXA) Antibody
357.5€ (100 µg)
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name
Beta-Hexosaminidase Subunit Alpha (HEXA) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx146346
tested applications
ELISA, WB, IHC
Description
HEXA Antibody is a Rabbit Polyclonal against HEXA.
Documents del producto
Instrucciones
Data sheet
Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Beta-Hexosaminidase Subunit Alpha (HEXA) |
| Host | Rabbit |
| Reactivity | Human |
| Recommended Dilution | ELISA: 1/20000 - 1/80000, WB: 1/500 - 1/2000, IHC: 1/100 - 1/200. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified by antigen affinity column chromatography. |
| Size 1 | 100 µg |
| Size 2 | 1 mg |
| Form | Lyophilized |
| Tested Applications | ELISA, WB, IHC |
| Buffer | Prior to lyophilization: 1% BSA and 0.02% NaN3. |
| Availability | Shipped within 7-15 working days. |
| Storage | Store at -20 °C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| NCBI Accession | BC018927 |
| Alias | N-acetyl-beta-glucosaminidase subunit alpha,TSD |
| Background | Antibody anti-HEXA |
| Status | RUO |
| Note | Concentration: Lyophilized form: Not applicable. After reconstitution: 1 mg/ml. - |
Descripción
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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