Definitions of N Acetyl beta D hexosaminidase

  1. 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. Deficiency of this isoenzyme causes TAY- SACHS DISEASE. Hexosaminidase B cleaves all the above substrates except GM2. A deficiency of both A and B causes SANDHOFF DISEASE. The enzyme has also been used as a tumor marker to distinguish between malignant and benign disease. EC 3. 2. 1. 52.