cd16147

glucosamine (N-acetyl)-6-sulfatase(G6S, GNS) AND sulfatase 1(SULF1)

CDD entry
Member databaseCDD
CDD typedomain
Short nameG6S
SetALP_like

Description

N-acetylglucosamine-6-sulfatase also known as glucosamine (N-acetyl)-6-sulfatase hydrolyzes of the 6-sulfate groups of the N-acetyl-D-glucosamine 6-sulfate units of heparan sulfate and keratan sulfate. Deficient of N-acetylglucosamine-6-sulfatase results in disease of Sanfilippo Syndrome type IIId or Mucopolysaccharidosis III (MPS-III), a rare autosomal recessive lysosomal storage disease. SULF1 encodes an extracellular heparan sulfate endosulfatase, that removes 6-O-sulfate groups from heparan sulfate chains of heparan sulfate proteoglycans (HSPGs).
[7, 2, 1, 4, 3, 9, 8, 6, 5]

References

1.Cloning of a mucin-desulfating sulfatase gene from Prevotella strain RS2 and its expression using a Bacteroides recombinant system. Wright DP, Knight CG, Parkar SG, Christie DL, Roberton AM. J Bacteriol 182, 3002-7, (2000). PMID: 10809675

2.Purification and characterization of N-acetylglucosamine-6-sulfate sulfatase from bovine kidney: evidence for the presence of a novel endosulfatase activity. Shilatifard A, Cummings RD. Biochemistry 33, 4273-82, (1994). PMID: 8155645

3.Crystal structure of an enzyme-substrate complex provides insight into the interaction between human arylsulfatase A and its substrates during catalysis. von Bulow R, Schmidt B, Dierks T, von Figura K, Uson I. J. Mol. Biol. 305, 269-77, (2001). View articlePMID: 11124905

4.Structure, function, and evolution of phosphoglycerate mutases: comparison with fructose-2,6-bisphosphatase, acid phosphatase, and alkaline phosphatase. Jedrzejas MJ. Prog. Biophys. Mol. Biol. 73, 263-87, (2000). View articlePMID: 10958932

5.Sulfatases and human disease. Diez-Roux G, Ballabio A. 6, 355-79, (2005). View articlePMID: 16124866

6.Sulfatase activities towards the regulation of cell metabolism and signaling in mammals. Buono M, Cosma MP. Cell. Mol. Life Sci. 67, 769-80, (2010). View articlePMID: 20165970

7.N-Acetylglucosamine-6-sulfate sulfatase from human urine. Basner R, Kresse H, von Figura K. J Biol Chem 254, 1151-8, (1979). PMID: 762121

8.The sulfatase gene family. Parenti G, Meroni G, Ballabio A. Curr. Opin. Genet. Dev. 7, 386-91, (1997). View articlePMID: 9229115

9.Three-dimensional structures of sulfatases. Ghosh D. Meth. Enzymol. 400, 273-93, (2005). View articlePMID: 16399355

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