Mouse Hexosaminidase B Beta (HEXb) ELISA Kit-Sandwich

The beta-hexosaminidase subunit beta catalyzes the hydrolysis of non-reducing end N-acetyl-D-hexosamine and/or sulfated N-acetyl-D-hexosamine from glycoconjugates, including the oligosaccharide components of proteins, neutral glycolipids, and certain mucopolysaccharides. While isozyme B does not hydrolyze all these substrates, it efficiently breaks down neutral oligosaccharides. Isozyme A is uniquely responsible for degrading GM2 gangliosides in the presence of GM2 activator protein (GM2A). In mice, the β-hexosaminidase β subunit (Hexb) is crucial for GM2 ganglioside degradation, and its functional defects can lead to severe neurodegenerative diseases like Sandhoff disease. Using gene knockout technology, researchers have developed HEXb knockout mouse models that accumulate significant quantities of GM2 ganglioside in the brain, displaying pathological features similar to human Sandhoff disease.