Arylsulfatase B

1FSU41111881ENSG00000113273ENSMUSG00000042082P15848P50429NM_000046NM_198709NM_009712NP_000037NP_942002NP_033842Arylsulfatase B (N-acetylgalactosamine-4-sulfatase, chondroitinsulfatase, chondroitinase, acetylgalactosamine 4-sulfatase, N-acetylgalactosamine 4-sulfate sulfohydrolase, EC 3.1.6.12) is an enzyme associated with mucopolysaccharidosis VI (Maroteaux–Lamy syndrome).

ARSB does this by breaking down glycosaminoglycans (GAGs), which are large sugar molecules in the body.

Used as a pharmaceutical drug, the enzyme is known under the International Nonproprietary Name galsulfase and is sold under the brand name Naglazyme.

[8][9][10] Galsulfase was approved for medical use in the United States in May 2005 and in European Union in January 2006.

Due to the complexity of arylsulfatase B's secondary structure, many hydrophobic and hydrophilic regions are present, as demonstrated by the Kyte-Doolittle hydropathy plot: Galsulfase is used to treat adults and children who have mucopolysaccharidosis VI (MPS VI or Maroteaux-Lamy syndrome).

[10] This causes the signs of the disease, the most noticeable being a short body, a large head and difficulty moving about.

The studies showed that decrease of ARSB leads to increase in free galectin-3, which attaches more strongly to less sulfated chondroitin 4-sulfate.

[22] Reduced sulfate availability due to impaired activity of ARSB has been linked to increased aerobic glycolysis, as shown by an increase in NADH and NADPH, reduced oxygen consumption, increased extracellular acidification and serum lactate, and a decline in mitochondrial membrane potential in ARSB-silenced cells and ARSB-null mouse tissues.

Kyte-Doolittle hydropathy plot: enlarge for better viewing