Mouse Hexosaminidase A Alpha (HEXa) ELISA Kit-Sandwich

Beta-hexosaminidase subunit alpha functions to hydrolyze the non-reducing end N-acetyl-D-hexosamine and/or sulfated N-acetyl-D-hexosamine in glycoconjugates, such as oligosaccharide moieties from proteins and neutral glycolipids, as well as certain mucopolysaccharides. The isozyme S is as active as isozyme A on anionic bis-sulfated glycans, including the chondroitin-6-sulfate trisaccharide (C6S-3), dermatan sulfate pentasaccharide, and sulfated glycosphingolipid SM2. In mice, the function of Hexa (HEX6) is primarily associated with the α subunit of β-galactosylceramidase (Hexosaminidase A). When the HEX6 gene is either lost or functions abnormally in mice, it results in a neurodegenerative condition similar to Tay-Sachs disease, which is marked by the accumulation of GM2 ceramide in neurons, ultimately leading to neurological dysfunction and death.