idu·ron·ate-2-sul·fa·tase (i″du-ronґāt sulґfə-tās) [EC] a lysosomal enzyme of the hydrolase class that catalyzes the cleavage of sulfate groups from the L-iduronate residues of dermatan sulfate and heparan sulfate, a step in the degradation of these glycosaminoglycans. Deficiency of the enzyme, an X-linked recessive trait, results in Hunter syndrome (mucopolysaccharidosis II).

Medical dictionary. 2011.

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  • Iduronate-2-sulfatase — is a sulfatase enzyme associated with Hunter syndrome. PBB Summary section title = summary text = Iduronate 2 sulfatase is required for the lysosomal degradation of heparan sulfate and dermatan sulfate. Mutations in this X chromosome gene that… …   Wikipedia

  • Sulfatase — Pfam box Symbol = Sulfatase Name = width =200 caption =Steryl sulfatase Pfam= PF00884 InterPro= IPR000917 SMART= PROSITE = PDOC00117 SCOP = 1auk TCDB = OPM family= 24 OPM protein= 1p49 PDB=PDB3|1fsu :43 486 PDB3|1hdhB:3 501 PDB3|1p49A:25… …   Wikipedia

  • iduronate — The salt or ester of iduronic acid. i. sulfatase an enzyme required for the desulfation of 2 sulfate i. residues in heparan sulfate. It is also required in dermatan sulfate degradation; Hunter syndrome is associated with a deficien …   Medical dictionary

  • Galactosamine-6 sulfatase — Galactosamine (N acetyl) 6 sulfate sulfatase Identifiers Symbols GALNS; FLJ17434; FLJ42844; FLJ98217; GALNAC6S; GAS; MPS4A External IDs …   Wikipedia

  • N-acetylglucosamine-6-sulfatase — glucosamine (N acetyl) 6 sulfatase (Sanfilippo disease IIID) Identifiers Symbol GNS Entrez 2799 HUGO …   Wikipedia

  • Hunter syndrome — Classification and external resources ICD 10 E76.1 ICD 9 277.5 …   Wikipedia

  • Arylsulfatase B — Crystallographic structure of putative tetrameric arylsulfatase from Escherichia coli.[1] Identifiers …   Wikipedia

  • List of EC numbers (EC 3) — This list contains a list of EC numbers for the third group, EC 3, hydrolases, placed in numerical order as determined by the Nomenclature Committee of the International Union of Biochemistry and Molecular Biology.EC 3.1: Acting on Ester BondsEC… …   Wikipedia

  • Hydrolase — Cristal de lysozyme Les hydrolases constituent une classe d enzymes qui catalysent les réactions d hydrolyse de molécules suivant la réaction générale : R R + H2O ⇌ R OH + R H On y trouve par exemple les estérases, qui hydrolysent les …   Wikipédia en Français

  • EC 3.1 — Le groupe EC3.1 est un groupe d hydrolases rassemblant les estérases, c est à dire les enzymes catalysant la réaction générale : R O R + H2O ⇌ R OH + R OH où R représente un acide quelconque et R son substrat. En fonction du milieu, l ester… …   Wikipédia en Français

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