Target intelligence / Profile preview

N-acetylgalactosamine 4-sulfatase (ARSB)

Target
ARSB
Molecular classification
Enzyme, Sulfatase, Lysosomal enzyme
01

Overview

N-acetylgalactosamine 4-sulfatase (ARSB) is a lysosomal enzyme that hydrolyzes the 4-sulfate groups from N-acetyl-D-galactosamine 4-sulfate units in chondroitin sulfate and dermatan sulfate. ARSB deficiency causes Mucopolysaccharidosis VI (MPS VI). Recombinant ARSB (galsulfase) is used as enzyme replacement therapy for MPS VI. ARSB also has roles in tumor suppression and cell signaling.

Other names
Arylsulfatase Bchondroitinsulfataseacetylgalactosamine 4-sulfatase
02

Mechanism of action

Enzyme replacement therapy; provides functional ARSB to degrade accumulated glycosaminoglycans

03

Biological functions

Glycosaminoglycan catabolismHydrolysis of 4-sulfate groups from N-acetyl-D-galactosamine 4-sulfateExtracellular matrix turnoverTumor suppressionRegulation of cell signaling
04

Disease associations

Mucopolysaccharidosis VI (Maroteaux-Lamy syndrome)Lysosomal storage disorderCancer (e.g., colon, breast, prostate)
05

Safety considerations

Infusion-related reactions (Galsulfase)Potential immunogenicity (Galsulfase)
06

Interacting drugs

Galsulfase (Naglazyme®)
07

Biomarkers

Urinary GAG levels (MPS VI)ARSB expression/activity (Cancer prognosis)Versican levels (Cancer microenvironment)

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