HEXA Antibody (Clone AT20F1)

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.
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Properties

Data Sheet Click for Datasheet
Catalog Number MA01307
Size 100 µL
Host Type Mouse
Immunogen Recombinant human HEXA (89-529aa) purified from E. coli
Specificity
Isotype IgG2a, l
Reacitivity Human
Clone Anti-human HEXA mAb, clone AT20F1, is derived from hybridization of mouse F0 myeloma cells with spleen cells from BALB/c mice immunized with a recombinant human HEXA protein.
Uniprot
Concentration 1 mg/ml
Dilution
Formulation Liquid. In Phosphate-Buffered Saline (pH 7.4) with 0.02% Sodium Azide, 10% Glycerol.
Application ELISA, WB.
Other Names beta-hexosaminidase subunit alpha, TSD, hexosaminidase A.
Storage Lyophilized product: 5 years at 2 - 8°C; Solution: 2 years at -20°C.
Postscript For research use only, not for use in diagnostic procedures.

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