Beta-Hexosaminidase Subunit Alpha (HEXA) Antibody
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Description
This gene encodes the alpha subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines.Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes.Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases.Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses.Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I).
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Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Beta-Hexosaminidase Subunit Alpha (HEXA) |
| Host | Mouse |
| Reactivity | Human |
| Recommended Dilution | ELISA: 1/10000, WB: 1/500 - 1/2000, FCM: 1/200 - 1/400. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Monoclonal |
| Conjugation | Unconjugated |
| Isotype | IgG2b |
| Clone ID | L575 |
| Purification | Purified from ascites by Protein G chromatography. |
| Size 1 | 15 µg |
| Size 2 | 100 µg |
| Form | Liquid |
| Tested Applications | ELISA, WB, FCM |
| Buffer | PBS, containing 0.05% sodium azide. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| Gene ID | 3073, 15211, 300757 |
| Alias | N-acetyl-beta-glucosaminidase subunit alpha,TSD |
| Background | Antibody anti-HEXA |
| Status | RUO |
| Note | Concentration: 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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