Target intelligence / Profile preview

Beta-N-acetylhexosaminidase (HEX)

Target
HEX
Molecular classification
Enzyme, Glycoside Hydrolase
01

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.

Other names
β-N-Acetyl-D-hexosaminideN-acetylhexosaminohydrolaseHexosaminidase AHexosaminidase BHexosaminidase Sβ-D-N-acetylhexosaminidaseβ-N-acetylglucosaminidase
02

Mechanism of action

Hydrolyzes terminal N-acetyl-D-glucosamine or galactosamine residues from glycoconjugates

03

Biological functions

Lysosomal degradationHydrolysis of glycoconjugatesChitin degradation
04

Disease associations

Tay-Sachs diseaseSandhoff diseaseLysosomal storage disorders
05

Safety considerations

Deficiency leads to severe neurodegenerative diseases
06

Biomarkers

Elevated levels in relapse during alcoholism treatmentDeficiency indicates lysosomal storage diseases

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