EC Number   |
Natural Substrates |
|---|
   3.1.6.12 | chondroitin 4-sulfate + H2O |
- |
| 3.1.6.12 | chondroitin sulfate + H2O |
- |
| 3.1.6.12 | chondroitin-4-sulfate + H2O |
arylsulfatase B removes 4-sulfate groups from the sulfated glycosaminoglycans chondroitin-4-sulfate and dermatan sulfate |
| 3.1.6.12 | dermatan sulfate + H2O |
arylsulfatase B removes 4-sulfate groups from the sulfated glycosaminoglycans chondroitin-4-sulfate and dermatan sulfate |
| 3.1.6.12 | dermatan sulfate + H2O |
- |
| 3.1.6.12 | more |
mucopolysaccharidosis type VI is an autosomal recessive lysosomal storage disorder caused by the deficiency of N-acetylgalactosamine-4-sulfatase. Mutations in the N-acetylgalactosamine-4-sulfatase gene are responsible for 4S deficiency, which leads to the intralysosomal storage of partially degraded glycosaminoglycan, dermatan sulfate, and chondroitin 4-sulfate |
| 3.1.6.12 | more |
the enzyme is required for the degradation of the glycosaminoglycan substrates dermatan and chondroitin sulfate. A 4-sulfatase deficiency results in the accumulation of undegraded substrate and causes the severe lysosomal storage disorder mucopolysaccharidosis type VI or Maroteaux-Lamy syndrome. A wide variation in clinical severity is observed between MPS VI patients and reflects the number of different 4-sulfatase mutations that can cause the disorder.Y210C is detected in about 10% of the MPS VI patients |
| 3.1.6.12 | more |
ARSB removes 4-sulfate groups from the nonreducing end of chondroitin-4-sulfate and dermatan sulfate |
| 3.1.6.12 | more |
ASB is a lysosomal exohydrolase, cleaving the 4-sulfate from the N-acetylgalactosamine-4-sulfate residue at the nonreducing terminal of glycosaminoglycan structures |
| 3.1.6.12 | N-acetyl-D-galactosamine 4-sulfate units + H2O |
acts also on N-acetylglucosamine 4-sulfate |
