ARSB hydrolyses C4S/C6S chains

Stable Identifier
Reaction [transition]
Homo sapiens
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Arylsulfatase B using calcium cofactor (ARSB:Ca2+) hydrolyses sulfate from N-acetylgalactosamine 4-sulfate (or 6-sulfate) units (GalNAc 4-sulfate or GalNAc 6-sulfate) within chondroitin sulfate (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
Participant Of
Catalyst Activity
Catalyst Activity
N-acetylgalactosamine-4-sulfatase activity of ARSB:Ca2+ [lysosomal lumen]
Physical Entity
Orthologous Events
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