Boster Immunoleader

antibody

Anti-HEXA Antibody

PA1787-2

100 ug/vial

200 USD

polyclonal

rabbit

Anti-HEXA Antibody

PA1787-2

Polyclonal

Rabbit IgG polyclonal antibody for Beta-hexosaminidase subunit alpha(HEXA) detection. Tested with WB in Human.

200 USD

Rabbit IgG

100 ug/vial

Lyophilized

No cross reactivity with other proteins.

Reacts with: mouse

WB

Concentration: 0.1-0.5μg/ml; Tested Species: Human

Tested Species: In-house tested species with positive results. Predicted Species: Species predicted to be fit for the product based on sequence similarities. Other applications have not been tested. Optimal dilutions should be determined by end users.

A synthetic peptide corresponding to a sequence at the C-terminus of human HEXA(513-529aa QAQPLNVGFCEQEFEQT), different from the related mouse sequence by three amino acids, and from the related rat sequences by four amino acids.

Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3.

Immunogen affinity purified.

Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

At -20˚C for one year. After reconstitution, at 4˚C for one month. It can also be aliquotted and stored frozen at -20˚C for a longer time. Avoid repeated freezing and thawing.

Boster provides a series of assays reacted with primary antibodies. Antibody can be supported by chemiluminescence kit EK1002 in WB.

HEXA(hexosaminidase A(alpha polypeptide)) is an enzyme that in humans is encoded by the HEXA gene. Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines The HEXA gene encodes the alpha subunit of hexosaminidase A, a lysosomal enzyme involved in the breakdown of gangliosides. The HEXA gene is mapped on 15q23. Even though the alpha and beta subunits of hexosaminidase A can both cleave GalNAc residues, only the alpha subunit is able to hydrolyze GM2 gangliosides. The alpha subunit contains a key residue, Arg-424, which is essential for binding the N-acetyl-neuramanic residue of GM2 gangliosides. Chimeric constructs were expressed in HeLa cells and selected constructs were produced in the baculovirus expression system to determine their ability to degrade GM2 ganglioside in the presence of GM2 activator protein. Their results allowed them to define 2 noncontiguous sequences in the alpha subunit(amino acids 1-191 and 403-529) which, when substituted into analogous positions in the beta subunit, conferred activity against the sulfated substrate.

1. Akli, S., Chomel, J.-C., Lacorte, J.-M., Bachner, L., Poenaru, A., Poenaru, L. Ten novel mutations in the HEXA gene in non-Jewish Tay-Sachs patients. Hum. Molec. Genet. 2: 61-67, 1993. 2. Beutler, E., Kuhl, W., Comings, D. Hexosaminidase isozyme in type O Gm2 gangliosidosis (Sandhoff-Jatzkewitz disease). Am. J. Hum. Genet. 27: 628-638, 1975. 3. Chern, C. J., Beutler, E., Kuhl, W., Gilbert, F., Mellman, W. J., Croce, C. M. Characterization of heteropolymeric hexosaminidase A in human x mouse hybrid cells. Proc. Nat. Acad. Sci. 73: 3637-3640, 1976.

HEXA

Beta-hexosaminidase subunit alpha

hexosaminidase A(alpha polypeptide)

Beta hexosaminidase alpha chain precursor antibody Beta hexosaminidase subunit alpha antibody Beta N acetylhexosaminidase antibody Beta N acetylhexosaminidase subunit alpha antibody Beta-hexosaminidase subunit alpha antibody Beta-N-acetylhexosaminidase subunit alpha antibody Hexa antibody HEXA_HUMAN antibody Hexosaminidase A(alpha polypeptide) antibody Hexosaminidase A alpha polypeptide antibody Hexosaminidase A antibody Hexosaminidase subunit A antibody MGC99608 antibody N acetyl beta glucosaminidase antibody N acetyl beta glucosaminidase subunit alpha antibody N-acetyl-beta-glucosaminidase subunit alpha antibody TSD antibody

P06865