218222
HEXA (Hexosaminidase A, beta-Hexosaminidase A)
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£438.00
- SKU:
- 218222
- Reinheit:
- Purified by Protein A affinity chromatography.
- Lagerbedingungen:
- 4°C/-20°C
- Hersteller:
- United States Biological
- Host:
- Mouse
- Immunogen:
- Recombinant protein corresponding to aa89-529 from human HEXA expressed in E. coli.
- Klon:
- 15B26
- Format:
- Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 10% glycerol.
- Weitere Details:
- HEXA (Hexosaminidase A), also designated beta-Hexosaminidase A, is responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. A mutation in the a subunit of hexosaminidase is the cause of Tay-Sachs disease (TSD), also known as GM2-gangliosidosis type I. TSD is a fatal autosomal recessive lysosomal storage disease of the central nervous system (CNS) caused by insufficient activity of the HEXA enzyme that results in a failure to process GM2 gangliosides. The accumulation of GM2 ganglioside in the absence of HEXA activity causes progressive destruction of the CNS. Applications: Suitable for use in ELISA and Western Blot. Other applications not tested. Recommended Dilution: Optimal dilutions to be determined by the researcher. Hybridoma: F0 myeloma cells with spleen cells from Balb/c mice. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
- Versandbedingungen:
- Blue Ice