Anti-HEXA Antibody Picoband®

SKU: A00692-1

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,ICC,IF,ELISA

Application Details: Western blot, 0.1-0.25 μg/ml, Human, Mouse, Rat
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: HEXA

Specificity:

Background: 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.

Immunogen: E.coli-derived human HEXA recombinant protein (Position: A135-H179).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 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.

Uniprot ID: P06865

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 60 kDa

Calculated Molecular Weight:

Gene ID: 3073

Protein Name: Beta-hexosaminidase subunit alpha

Gene Full Name: hexosaminidase subunit alpha

Synonyms: HEXA; Hexosaminidase subunit A; TSD