Reactome | ARSB hydrolyses DS

ARSB hydrolyses DS

Stable Identifier

R-HSA-1606789

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lysosomal lumen

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Arylsulfatase B (ARSB) hydrolyses sulfate from N-acetylgalactosamine 4-sulfate units within dermatan sulfate (DS; Gorham & Cantz 1978). The conversion to 3-oxoalanine (formylglycine, FGly) of a cysteine residue in eukaryotes, is critical for catalytic activity, based on similarity to the prototypical arylsulfatase ARSA (Chruszcz et al. 2003, Lukatela et al. 1998). Defects in ARSB are the cause of mucopolysaccharidosis type VI (MPSVI) (MIM:253200, also called Maroteaux-Lamy syndrome (Wicker et al. 1991). ARSB activity is defective in multiple sulfatase deficiency (MSD) (MIM:272200) (Schmidt et al. 1995).

Literature References

PubMed ID	Title	Journal	Year
738706	Arylsulphatase B, an exo-sulphatase for chondroitin 4-sulphate tetrasaccharide Gorham, SD, Cantz, M	Hoppe Seylers Z Physiol Chem	1978
9521684	Crystal structure of human arylsulfatase A: the aldehyde function and the metal ion at the active site suggest a novel mechanism for sulfate ester hydrolysis Lukatela, G, Krauss, N, Theis, K, Selmer, T, Gieselmann, V, von Figura, K, Saenger, W	Biochemistry	1998
7628016	A novel amino acid modification in sulfatases that is defective in multiple sulfatase deficiency Schmidt, B, Selmer, T, Ingendoh, A, von Figura, K	Cell	1995
12888274	Crystal structure of a covalent intermediate of endogenous human arylsulfatase A Chruszcz, M, Laidler, P, Monkiewicz, M, Ortlund, E, Lebioda, L, Lewinski, K	J Inorg Biochem	2003