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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.

Year introduced: 2008(1988)

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Tree Number(s): D08.811.277.450.483.180

MeSH Unique ID: D001619

Registry Number: EC

Entry Terms:

  • beta N Acetylhexosaminidases
  • N-Acetyl-beta-D-hexosaminidase
  • N Acetyl beta D hexosaminidase
  • beta-N-Acetyl-hexosaminidase
  • beta N Acetyl hexosaminidase
  • beta-N-Acetylhexosaminidase
  • beta N Acetylhexosaminidase
  • beta-Hexosaminidase
  • beta Hexosaminidase
  • beta-N-Acetyl-D-hexosaminidase
  • beta N Acetyl D hexosaminidase

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