Beta-hexosaminidase subunit alpha
Function
Hydrolyzes the non-reducing end N-acetyl-D-hexosamine and/or sulfated N-acetyl-D-hexosamine of glycoconjugates, such as the oligosaccharide moieties from proteins and neutral glycolipids, or from certain mucopolysaccharides. The isozyme S is as active as the isozyme A on the anionic bis-sulfated glycans, the chondroitin-6-sulfate trisaccharide (C6S-3), and the dermatan sulfate pentasaccharide, and the sulfated glycosphingolipid SM2. The isozyme B does not hydrolyze each of these substrates, however hydrolyzes efficiently neutral oligosaccharide. Only the isozyme A is responsible for the degradation of GM2 gangliosides in the presence of GM2A.
Sequence Similarities
Belongs to the glycosyl hydrolase 20 family.
Tissue Specificity
Ubiquitous. Most abundant in testis, adrenal, epididymis and heart. Low levels seen in the liver.
Cellular localization
- Lysosome
Alternative names
Beta-hexosaminidase subunit alpha, Beta-N-acetylhexosaminidase subunit alpha, N-acetyl-beta-glucosaminidase subunit alpha, Hexosaminidase subunit A, Hexa