Arylsulfatase B

• OMIM: 611 542
• UniProt: P15848

Arylsulfatase B (including: N-acetylgalactosamine -4- sulfate sulfatase ) is the enzyme in the animal, the sulfate of mucopolysaccharides of the chondroitin sulfate and dermatan cleaved. The reduction of these cartilage -forming macromolecules takes place in the lysosomes of cells. Is in humans, the enzyme activity is not or too little present, which can be caused by mutations in the ARSB gene occurs by the accumulation of mucopolysaccharides in the lysosomes to a metabolic disorder of mucopolysaccharidosis type VI ( Maroteaux -Lamy syndrome) with skeletal malformations. This can be treated successfully since 2006 using an enzyme replacement therapy with artificially produced enzyme ( Galsulfase, Biomarin Pharmaceutical).

Arylsulfatase B belongs to the sulfatase, enzymes cleave the sulfate. The formylation of the cysteine ​​91 to Ketoalanin ( post-translational modification ), is critical to the capture of the calcium - cofactor and enzyme activity. A defect in this process affects multiple sulfatase and leads to multiple sulfatase.

The effect of antimalarial chloroquine seems partly to be due to an inhibition of arylsulfatase B. Outside of sulfatase activity, the enzyme seems to have regulatory effects on interleukin-8 pathway and on the epithelial cell migration.