Beta N acetylhexosaminidase

An enzyme hydrolyzing terminal non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides. It acts on GLUCOSIDES; GALACTOSIDES; and several OLIGOSACCHARIDES. Hexosaminidase A cleaves GM2, GA2, globosides, and hexosamine oligosaccharides. Hexosaminidase B cleaves all the above substrates except GM2. Deficiency of Hexosaminidase A causes TAY-SACHS DISEASE, while deficiency of both A and B isozymes causes SANDHOFF DISEASE. The enzyme has also been used as a tumor marker to distinguish between malignant and benign disease.