Chemicals and Drugs

Hexosaminidase A

A mammalian beta-hexosaminidase isoform that is a heteromeric protein comprized of both hexosaminidase alpha and hexosaminidase beta subunits. Deficiency of hexosaminidase A due to mutations in the gene encoding the hexosaminidase alpha subunit is a case of TAY-SACHS DISEASE. Deficiency of hexosaminidase A and HEXOSAMINIDASE B due to mutations in the gene encoding the hexosaminidase beta subunit is a case of SANDHOFF DISEASE.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

A mammalian beta-hexosaminidase isoform that is a heteromeric protein comprized of both hexosaminidase alpha and hexosaminidase beta subunits. Deficiency of hexosaminidase A due to mutations in the gene encoding the hexosaminidase alpha subunit is a case of TAY-SACHS DISEASE. Deficiency of hexosaminidase A and HEXOSAMINIDASE B due to mutations in the gene encoding the hexosaminidase beta subunit is a case of SANDHOFF DISEASE.

MeSH Record

Classification

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See Also

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Synonyms

3 entry terms
  • Hex A
  • beta-N-Acetylhexosaminidase A
  • beta N Acetylhexosaminidase A

MeSH Record

Aspects Covered

28 allowable subheadings

Indexed with the subheadings administration & dosage, adverse effects, analysis, antagonists & inhibitors, biosynthesis, blood, cerebrospinal fluid, chemical synthesis, chemistry, classification, 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

for deficiency consider: SANDHOFF DISEASE or TAY-SACHS DISEASE

MeSH Record

History Note

2008(1987); for HEXOSAMINIDASE A use BETA-N-ACETYLHEXOSAMINIDASE 1987-2007

MeSH Record

Previous Indexing

  • beta-N-Acetylhexosaminidases (1999-2007)

MeSH Hierarchy

Tree Number

  1. D08.811w.277w.450w.483w.180.750

AMA Style

References

  1. National Library of Medicine. Hexosaminidase A. Medical Subject Headings (MeSH). 2026. Unique ID D054818. http://id.nlm.nih.gov/mesh/2026/D054818
  2. Hexosaminidase A. In: Wikidata. https://www.wikidata.org/wiki/Q76876844