Beta-Hexosaminidase Subunit Alpha (HEXA) Antibody (Biotin)
169€ (20 µg)
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Name
Beta-Hexosaminidase Subunit Alpha (HEXA) Antibody (Biotin)
Category
Primary Antibodies
Provider
Abbexa
Reference
abx334835
Tested Applications
ELISA
Description
HEXA Antibody (Biotin) is a Rabbit Polyclonal against HEXA.
Documentos del producto
Instrucciones
Data sheet
Especificaciones del producto
| Category | Primary Antibodies |
| Immunogen Target | Target: Beta-Hexosaminidase Subunit Alpha (HEXA) Immunogen: Recombinant Human Beta-hexosaminidase subunit alpha protein (89-529AA). |
| Host | Rabbit |
| Reactivity | Human |
| Recommended Dilution | Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Biotin |
| Isotype | IgG |
| Purity | > 95% |
| Purification | Purified by Protein G. |
| Size 1 | 20 µg |
| Size 2 | 50 µg |
| Size 3 | 100 µg |
| Size 4 | 200 µg |
| Size 5 | 1 mg |
| Form | Liquid |
| Tested Applications | ELISA |
| Buffer | 0.01 M PBS, pH 7.4, 0.03% Proclin-300 and 50% Glycerol. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid exposure to light. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | P06865 |
| Gene ID | 3073 |
| Alias | N-acetyl-beta-glucosaminidase subunit alpha,TSD |
| Background | Antibody anti-HEXA |
| Status | RUO |
| Note | THIS PRODUCT IS FOR RESEARCH USE ONLY. NOT FOR USE IN DIAGNOSTIC, THERAPEUTIC OR COSMETIC PROCEDURES. NOT FOR HUMAN OR ANIMAL CONSUMPTION. |
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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