ARSB hydrolyses DS

Stable Identifier
R-HSA-1606789
Type
Reaction
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
General
SVG |   | PPTX  | SBGN
Click the image above or here to open this reaction in the Pathway Browser
The layout of this reaction may differ from that in the pathway view due to the constraints in pathway layout

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
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

Krauss, N, Selmer, T, Gieselmann, V, Saenger, W, von Figura, K, Lukatela, G, Theis, K

Biochemistry 1998
738706 Arylsulphatase B, an exo-sulphatase for chondroitin 4-sulphate tetrasaccharide

Cantz, M, Gorham, SD

Hoppe Seylers Z Physiol Chem 1978
7628016 A novel amino acid modification in sulfatases that is defective in multiple sulfatase deficiency

Selmer, T, Ingendoh, A, von Figura, K, Schmidt, B

Cell 1995
12888274 Crystal structure of a covalent intermediate of endogenous human arylsulfatase A

Lebioda, L, Lewinski, K, Laidler, P, Ortlund, E, Monkiewicz, M, Chruszcz, M

J Inorg Biochem 2003
Participants
Participates
Catalyst Activity

N-acetylgalactosamine-4-sulfatase activity of ARSB:Ca2+ [lysosomal lumen]

Orthologous Events
Authored
Reviewed
Created
Cite Us!