beta-N-Acetylhexosaminidase

Medical Dictionary -> beta-N-Acetylhexosaminidase

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.


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