Rat Beta-hexosaminidase Subunit Alpha, HEXA ELI

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.