Hexosaminidase A antibody (AA 135-179) (HRP)

Catalog No. ABIN7997481

This Rabbit Polyclonal antibody specifically detects Hexosaminidase A in WB, ELISA and IHC. It exhibits reactivity toward Human, Mouse and Rat.

Quick Overview for Hexosaminidase A antibody (AA 135-179) (HRP) (ABIN7997481)

Target: Hexosaminidase A (HEXA)

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Polyclonal

Conjugate: This Hexosaminidase A antibody is conjugated to HRP

Application: Western Blotting (WB), ELISA, Immunohistochemistry (IHC)

Product Details anti-Hexosaminidase A Antibody (HRP)

Binding Specificity: AA 135-179

Purpose: Anti-HEXA Antibody HRP Conjugated

Cross-Reactivity (Details): No cross-reactivity with other proteins.

Purification: Immunogen affinity purified.

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

Isotype: IgG

Application Details

Application Notes: Western blot, Optimal dilutions should be determined by end users. Immunohistochemistry (Paraffin-embedded Section), Optimal dilutions should be determined by end users. ELISA, Optimal dilutions should be determined by end users.

Restrictions: For Research Use only

Handling

Format: Liquid

Buffer: Each vial contains 50 % glycerol, 0.9 % NaCl, 0.2 % Na2HPO4.

Storage: -20 °C

Storage Comment: At -20°C for one year from date of receipt. Avoid repeated freezing and thawing.

Expiry Date: 12 months

Target Details for Hexosaminidase A

Target: Hexosaminidase A (HEXA)

Alternative Name: HEXA

Background:

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.

Gene Full Name: hexosaminidase subunit alpha

Gene ID: 3073

UniProt: P06865

Pathways: Sensory Perception of Sound, Glycosaminoglycan Metabolic Process