ENTRY EC 3.1.6.13
NAME Iduronate-2-sulfatase
Chondroitinsulfatase
CLASS Hydrolases
Acting on ester bonds
Sulfuric ester hydrolases
SYSNAME L-iduronate-2-sulfate 2-sulfohydrolase
REACTION Hydrolysis of the 2-sulfate groups of the L-iduronate 2-sulfate
units of dermatan sulfate, heparan sulfate and heparin
SUBSTRATE L-Iduronate 2-sulfate
H2O
Dermatan sulfate
Heparan sulfate
Hiparin
PRODUCT Sulfate
DISEASE MIM: 309900 Iduronate 2-sulfatase (Hunter syndrome);
Mucopolysaccharidosis II (3)
MOTIF PS: PS00149 G-Y-x-[ST]-x(2)-[LIVMA]-G-K-x(0,1)-[FYW]
PS: PS00523 [SAP]-[LIVMS]-[CS]-x-P-[STA]-R-x(2)-[LIVMF](2)-T-G
GENES HSA: IDS(SIDS)
DBLINKS University of Geneva ENZYME DATA BANK: 3.1.6.13
WIT (What Is There) Metabolic Reconstruction: 3.1.6.13
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