Chemicals and Drugs

beta-N-Acetylhexosaminidases

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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.

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Synonyms

11 entry terms
  • N-Acetyl-beta-D-hexosaminidase
  • beta-Hexosaminidase
  • beta-N-Acetyl-D-hexosaminidase
  • beta-N-Acetyl-hexosaminidase
  • beta-N-Acetylhexosaminidase
  • N Acetyl beta D hexosaminidase
  • beta Hexosaminidase
  • beta N Acetyl D hexosaminidase
  • beta N Acetyl hexosaminidase
  • beta N Acetylhexosaminidase
  • beta N Acetylhexosaminidases

MeSH Record

Aspects Covered

29 allowable subheadings

Indexed with the subheadings administration & dosage, adverse effects, analysis, antagonists & inhibitors, biosynthesis, blood, cerebrospinal fluid, chemical synthesis, chemistry, classification, deficiency, drug effects, economics, genetics, history, immunology, isolation & purification, metabolism, pharmacokinetics, pharmacology, physiology, poisoning, radiation effects, standards, supply & distribution, therapeutic use, toxicity, ultrastructure, urine.

MeSH Record

Indexing Annotation

do not confuse with ACETYLGLUCOSAMINIDASE; /defic: consider also TAY SACHS DISEASE and SANDHOFF DISEASE

MeSH Record

History Note

2008(1988)

MeSH Record

Previous Indexing

  • Glycoside Hydrolases (1966-1970)
  • Hexosaminidases (1971-1986)

MeSH Hierarchy

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AMA Style

References

  1. National Library of Medicine. beta-N-Acetylhexosaminidases. Medical Subject Headings (MeSH). 2026. Unique ID D001619. http://id.nlm.nih.gov/mesh/2026/D001619
  2. beta-N-Acetylhexosaminidases. In: Wikidata. https://www.wikidata.org/wiki/Q24745388