Definition: A hexosaminidase specific for non-reducing N-acetyl-D-hexosamine residues in N-acetyl-beta-D-hexosaminides. It acts on GLUCOSIDES; GALACTOSIDES; and several OLIGOSACCHARIDES. Two specific mammalian isoenzymes of beta-N-acetylhexoaminidase are referred to as HEXOSAMINIDASE A and HEXOSAMINIDASE B. Deficiency of the type A isoenzyme 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.
Other names beta-N-Acetylhexosaminidase; beta N Acetylhexosaminidases; beta N Acetylhexosaminidase; beta N Acetyl hexosaminidase; beta N Acetyl D hexosaminidase; beta Hexosaminidase; N Acetyl beta D hexosaminidase; beta-N-Acetyl-hexosaminidase; beta-N-Acetyl-D-hexosaminidase; beta-Hexosaminidase; N-Acetyl-beta-D-hexosaminidase