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Arylsulphatase B (None)
- Target
- None
- Molecular classification
- Enzyme, Sulfatase, Lysosomal enzyme
01
Overview
Arylsulphatase B (ARSB), also known as N-acetylgalactosamine-4-sulfatase, is a lysosomal enzyme responsible for the degradation of dermatan sulfate and chondroitin sulfate. Deficiency or mutations in ARSB lead to mucopolysaccharidosis type VI (MPS VI; Maroteaux-Lamy syndrome). Recombinant human arylsulfatase B (galsulfase) is used as an enzyme replacement therapy.
- Other names
- N-acetylgalactosamine-4-sulfatase
02
Mechanism of action
Enzyme replacement therapy
03
Biological functions
Hydrolyzes sulfates in lysosomesDegradation of dermatan sulfateDegradation of chondroitin sulfateTumor suppressor (potential)Regulation of transcriptional responsesRedox switch
04
Disease associations
Mucopolysaccharidosis type VI (MPS VI; Maroteaux-Lamy syndrome)Lysosomal storage disorderCancer (potential)Bone metabolism disordersCystic fibrosis (potential)
05
Safety considerations
Infusion-related reactions (with galsulfase)Potential immunogenicity (with galsulfase)
06
Interacting drugs
Galsulfase (Naglazyme)
07
Biomarkers
ARSB enzyme activityDermatan sulfate levelsChondroitin sulfate levelsOsteoclast marker (potential)
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