HEXA (Beta-N-acetylhexosaminidase subunit alpha)

HEXA(hexosaminidase A (alpha polypeptide)) is an enzyme that in humans is encoded by \nthe HEXA gene. Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of \nthe GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines The HEXA gene \nencodes the alpha subunit of hexosaminidase A , a lysosomal enzyme involved in the breakdown of \ngangliosides. The HEXA gene is mapped on 15q23. Even though the alpha and beta subunits of \nhexosaminidase A can both cleave GalNAc residues, only the alpha subunit is able to\nhydrolyze GM2 gangliosides. The alpha subunit contains a key residue, Arg-424, which is essential for \nbinding the N-acetyl-neuramanic residue of GM2 gangliosides. Chimeric constructs were expressed in \nHeLa cells and selected constructs were produced in the baculovirus expression system to determine \ntheir ability to degrade GM2 ganglioside in the presence of GM2 activator protein. Their results allowed \nthem to define 2 noncontiguous sequences in the alpha subunit (amino acids 1-191 and 403-529) which, \nwhen substituted into analogous positions in the beta subunit, conferred activity against the sulfated \nsubstrate.\n\nUniProt Number:\nP06865\n\nGene ID:\nHEXA\n\nApplications:\nSuitable for use in Western Blot and Immunohistochemistry (Paraffin).\n\nRecommended Dilution:\nOptimal dilutions to be determined by the researcher.\n\nStorage and Handling:\nStore at -20?C for one year. After reconstitution, store at 4?C for one month. Can also be aliquoted and stored frozen at -20?C for long term.\nAvoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

144657

Size

100ug

Applications

IHC, WB

Hosts

Rabbit

Reactivities

Hum, Mouse, Rat

Form

Supplied as a lyophilized powder. Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3. Reconstitution: Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

P Type

Pab

Purity

Purified by immunoaffinity chromatography.

Isotype

IgG

References

1. Akli, S., Chomel, J.-C., Lacorte, J.-M., Bachner, L., Poenaru, A., Poenaru, L. Ten novel mutations in \nthe HEXA gene in non-Jewish Tay-Sachs patients. Hum. Molec. Genet. 2: 61-67, 1993.\n2. Beutler, E., Kuhl, W., Comings, D. Hexosaminidase isozyme in type O Gm2 gangliosidosis \n(Sandhoff-Jatzkewitz disease). Am. J. Hum. Genet. 27: 628-638, 1975.\n3. Chern, C. J., Beutler, E., Kuhl, W., Gilbert, F., Mellman, W. J., Croce, C. M. Characterization of \nheteropolymeric hexosaminidase A in human x mouse hybrid cells. Proc. Nat. Acad. Sci. 73: \n3637-3640, 1976.

Additional Info

Recognizes human, mouse and rat HEXA. No crossreactivity with other proteins.