ENTRY EC 3.2.1.76
NAME L-Iduronidase
CLASS Hydrolases
Glycosidases
Hydrolysing O-glycosyl compounds
SYSNAME Glycosaminoglycan alpha-L-iduronohydrolase
REACTION Hydrolysis of unsulfated alpha-L-iduronosidic linkages in
dermatan sulfate
SUBSTRATE Dermatan sulfate
H2O
PRODUCT Dermatan sulfate
DISEASE MIM: 252800 Iduronidase, alpha-L-; Mucopolysaccharidosis Ih (3);
Mucopolysaccharidosis Is (3); Mucopolysaccharidosis
Ih/s (3)
MOTIF PS: PS01027 W-x-F-E-x-W-N-E-P-[DN]
GENES HSA: IDUA(Hs.89560)
DBLINKS University of Geneva ENZYME DATA BANK: 3.2.1.76
WIT (What Is There) Metabolic Reconstruction: 3.2.1.76
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