Arylsulfatase A hydrolyses sulfate from sulfatide to form cerebroside

Stable Identifier
R-HSA-1606807
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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Arylsulfatase A (ARSA) (Stein et al. 1989) hydrolyses a sulfatide (a cerebroside 3-sulfate) to form a cerebroside and sulfate. ARSA is present in the lysosomal lumen and comprises two chains, component B and C linked by disulphide bonds (Fujii et al. 1992). The conversion to 3-oxoalanine (formylglycine, FGly) of a cysteine residue is critical for catalytic activity in all eukaryotes (Chruszcz et al. 2003, Lukatela et al. 1998).
Defects in ARSA are a cause of leukodystrophy metachromatic (MLD) (MIM:250100), characterized by lysosomal storage of cerebroside-3-sulfate in neural and non-neural tissues (Gieselmann et al. 1991, Polten et al. 1991). Arylsulfatase A activity is reduced in multiple sulfatase deficiency (MSD) (MIM:272200), a disorder characterized by decreased activity of sulfatases. The defect is due to the lack of post-translational modification of the critical cysteine needed for activity (Schmidt et al. 1995).

Literature References
PubMed ID Title Journal Year
2562955 Cloning and expression of human arylsulfatase A

Stein, C, Gieselmann, V, Kreysing, J, Schmidt, B, Pohlmann, R, Waheed, A, Meyer, HE, O'Brien, JS, von Figura, K

J Biol Chem 1989
1670590 Molecular basis of different forms of metachromatic leukodystrophy

Polten, A, Fluharty, AL, Fluharty, CB, Kappler, J, von Figura, K, Gieselmann, V

N Engl J Med 1991
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
1352993 Proteolytic processing of human lysosomal arylsulfatase A

Fujii, T, Kobayashi, T, Honke, K, Gasa, S, Ishikawa, M, Shimizu, T, Makita, A

Biochim Biophys Acta 1992
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
1678251 Mutations in the arylsulfatase A pseudodeficiency allele causing metachromatic leukodystrophy

Gieselmann, V, Fluharty, AL, T√łnnesen, T, von Figura, K

Am J Hum Genet 1991
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
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
cerebroside-sulfatase activity of active ARSA:Ca2+ [lysosomal lumen]
Physical Entity
Activity
Orthologous Events
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Reviewed
Created
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