HEXA Antibody

Hexosaminidase A (HEXA) is an enzyme that plays a crucial role in the degradation of glycosphingolipids, specifically GM2 gangliosides, within lysosomes. This enzyme is composed of two subunits, alpha and beta, which are encoded by the HEXA and HEXB genes, respectively . The enzyme’s primary function is to hydrolyze terminal N-acetyl-D-hexosamine residues in GM2 gangliosides and globo-sphingolipids .

Deficiencies in HEXA activity lead to the accumulation of GM2 gangliosides, resulting in severe neurodegenerative disorders such as Tay-Sachs disease and Sandhoff disease . These lysosomal storage diseases are characterized by progressive neurological deterioration, leading to early mortality in affected individuals .

The mouse anti-human Hexosaminidase A antibody is a monoclonal antibody that specifically detects human HEXA in various applications, including Western blotting and immunohistochemistry . This antibody is produced by immunizing mice with recombinant human HEXA protein, followed by hybridoma technology to generate a specific monoclonal antibody .

1. Western Blotting: The mouse anti-human HEXA antibody is used to detect HEXA in cell lysates and tissue samples. It is particularly useful in identifying the presence and quantity of HEXA in different biological samples .
2. Immunohistochemistry: This antibody is employed to visualize HEXA expression in tissue sections, providing insights into the localization and distribution of HEXA within cells and tissues .

Research involving HEXA and its antibodies has significant implications for understanding and treating lysosomal storage diseases. By studying the expression and activity of HEXA, scientists can develop therapeutic strategies to enhance HEXA function and mitigate the effects of GM2 ganglioside accumulation .