Medical Dictionary

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N Acetyl beta D hexosaminidase

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.

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