Iduronate 2-sulfate sulfatase

This enzyme [EC 3.1.6.13], also known as iduronate-2-sulfate sulfatase and chondroitinsulfatase, catalyzes the... [Pg.359]

Timms KM, Lu F, Shen Y, Pierson CA, Muzny DM, Gu Y, Nelson DL, Gibbs RA. 130 kb of DNA sequence reveals two new genes and a regional duplication distal to the human iduronate-2-sulfate sulfatase locus. Genome Res 1995 5(l) 71-78. [Pg.633]

Eliahu, R., Sekeles, E., Cohen, R., and Bach, G., The correction of Hunter fibroblasts by enogenous iduronate sulfate sulfatase Biochemical and ultrastructural studies. Am. J. Hum. Genet. 33, 576-583 (1981). [Pg.190]

Sulfotransferases917 920a transfer sulfo groups to O and N atoms of suitable acceptors (reaction type ID, Table 10-1). Usually, transfer is from the "active sulfate," 3 -phosphoadenosine 5 -phosphosuIfate (PAPS),921 whose formation is depicted in Eq. 17-38. Sulfatases catalyze hydrolysis of sulfate esters. The importance of such enzymes is demonstrated by the genetic mucopolysaccharidoses. In four of these disease-specific sulfatases that act on iduronate sulfate, heparan N-sulfate, galactose-6-sulfate, or N-acetylglu-cosamine-4-sulfate are absent. Some of these, such as heparan N-sulfatase deficiency, lead to severe mental retardation, some cause serious skeletal abnormalities, while others are mild in their effects.922... [Pg.659]

MPII, Hunter Iduronic acid-O-2 sulfate sulfatase HS, DS... [Pg.237]

Dermatan sulfate, L-Iduronic acid heparan sulfate 4-sulfate sulfatase... [Pg.60]

J 11 Hunter (mild or severe) 309900 Dermatan sulfate Heparan sulfate Iduronate-2-sulfatase Xq28... [Pg.380]

Hurler-Scheie (MIM 252800) Hunter (MIM 309900) MPS II Iduronate sulfatase Dermatan sulfate, heparan sulfate... [Pg.546]

Hunter s syndrome MPS 11 Iduronate sulfatase Dermatan sulfate Heparan sulfate... [Pg.686]

This enzyme [EC 3.1.6.18], also known as glucuronate-2-sulfatase and chondro-2-sulfatase, catalyzes the hydrolysis of the 2-sulfate groups of the 2-O-sulfo-D-glucuro-nate residues of chondroitin sulfate, heparin, and hepari-tin sulfate. The enzyme does not act on iduronate 2-sulfate residues. [Pg.313]

The missing enzyme is a sulfatase for 2-sulfated iduronate residues.354 355 The diagram at the bottom of the page illustrates the need for both of these enzymes as well as three others in the degradation of dermatan 352/356/357... [Pg.1169]

MPS II (Hunter) Iduronate sulfatase Dermatan sulfate, heparan sulfate Skeletal, bone, organomegaly, neurological Mouse, dog Plasmid IM (Tomanin et al., 2002)... [Pg.250]

E. Since this patient is suspected of having Hunter syndrome, a deficiency in iduronate sulfatase, iduronate 2-sulfate would be expected to be present at the nonreducing end of glycosaminoglycans found in this patient s urine. A deficiency of iduronate sulfatase would prevent the sulfate ester bond of iduronate 2-sulfate residues from being hydrolyzed and further degradation of the glycosaminoglycan would be halted. [Pg.270]

Hunter Hurler + Scheie Maroteaux-Lamy Mucolipidosis Vli Sanfilippo A Sanfilippo B Sanfilippo D iduronate suifatase a-L-iduronidase A/-Acetylgalactosamine suifatase p-Glucuronidase Heparan sulfamidase A/-Acetylglucosaminidase A/-Acetylglucosamine 6-sulfatase Heparan sulfate. Dermatan sulfate Heparan sulfate. Dermatan sulfate Dermatan sulfate Heparan sulfate. Dermatan sulfate Heparan sulfate Heparan sulfate Heparin sulfate... [Pg.915]

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