Description
HEXA Antibody / Hexosaminidase A | RQ6641 | Gentaur US, UK & Europe Disrtribition
Family: Primary antibody
Formulation: 0.5mg/ml if reconstituted with 0.2ml sterile DI water
Format: Antigen affinity purified
Clone: N/A
Host Animal: Rabbit
Clonality: Polyclonal (rabbit origin)
Species Reactivity: Human, Mouse, Rat
Application: WB, IF, Direct ELISA
Buffer: Lyophilized from 1X PBS with 2% Trehalose
Limitation: This Hexosaminidase A antibody is available for research use only.
Purity: Antigen affinity purified
Description: 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: Recombinant human protein (amino acids A135-H179) was used as the immunogen for the Hexosaminidase A antibody.
Storage: After reconstitution, the Hexosaminidase A antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20 °C. Avoid repeated freezing and thawing.
