Cow Beta-Hexosaminidase Subunit Alpha (HEXA) ELISA Kit
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Description
Cow Beta-Hexosaminidase Subunit Alpha (HEXA) ELISA Kit is an ELISA Kit for the in vitro quantitative measurement of Cow Beta-hexosaminidase subunit alpha concentrations in tissue homogenates, cell lysates and other biological fluids.
Documents del producto
Product specifications
| Category | ELISA Kits |
| Immunogen Target | Beta-Hexosaminidase Subunit Alpha (HEXA) |
| Reactivity | Cow |
| Detection Method | Colorimetric |
| Assay Data | Quantitative |
| Test Range | 0.156 ng/ml - 10 ng/ml |
| Recommended Dilution | Optimal dilutions/concentrations should be determined by the end user. |
| Size 1 | 96 tests |
| Form | Lyophilized |
| Tested Applications | ELISA |
| Sample Type | Tissue homogenates, cell lysates and other biological fluids. |
| Availability | Shipped within 5-12 working days. The validity for this kit is 6 months. |
| Storage | Shipped at 4 °C. Upon receipt, store the kit according to the storage instruction in the kit's manual. |
| Dry Ice | No |
| UniProt ID | Q0V8R6 |
| Alias | N-acetyl-beta-glucosaminidase subunit alpha,TSD |
| Background | Elisa kits for HEXA |
| Status | RUO |
| Note | Validity: The validity for this kit is 6 months. This product is for research use only. The range and sensitivity is subject to change. Please contact us for the latest product information. For accurate results, sample concentrations must be diluted to mid-range of the kit. If you require a specific range, please contact us in advance or write your request in your order comments. Please note that our ELISA and CLIA kits are optimised for detection of native samples, rather than recombinant proteins. We are unable to guarantee detection of recombinant proteins, as they may have different sequences or tertiary structures to the native protein. |
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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