Gosset Molecular Classification
Enzyme, Glycoside Hydrolase
Other Names
β-N-Acetyl-D-hexosaminide, N-acetylhexosaminohydrolase, Hexosaminidase A, Hexosaminidase B, Hexosaminidase S, β-D-N-acetylhexosaminidase, β-N-acetylglucosaminidase
Disease Roles
Beta-N-acetylhexosaminidase Overview
Beta-N-acetylhexosaminidase is an exoglycosidase enzyme that catalyzes the hydrolysis of terminal, non-reducing β-N-acetylgalactosamine and β-N-acetylglucosamine residues from glycoconjugates. It plays a crucial role in the degradation of N-acetylated carbohydrates and glycoproteins by releasing N-acetylhexosamines. Deficiency in this enzyme leads to lysosomal storage disorders such as Tay-Sachs and Sandhoff diseases.
Mechanism of Action
Hydrolyzes terminal N-acetyl-D-glucosamine or galactosamine residues from glycoconjugates
Biological Functions
Lysosomal degradation
Hydrolysis of glycoconjugates
Chitin degradation
Disease Associations
Tay-Sachs disease
Sandhoff disease
Lysosomal storage disorders
Safety Considerations
- Deficiency leads to severe neurodegenerative diseases
Associated Biomarkers
| Biomarker |
|---|
| Elevated levels in relapse during alcoholism treatment |
| Deficiency indicates lysosomal storage diseases |