GALNS oligomer hydrolyses sulfate from Gal6S in keratan sulfate

Stable Identifier
Reaction [transition]
Homo sapiens
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N-acetylgalactosamine 6-sulfate sulfatase (GALNS) hydrolyses sulfate from galactose 6-sulfate units of keratan sulfate (KS, shown here) and sulfate from N-acetyl-D-galactosamine 6-sulfate units of chondroitin sulfate (CS, not shown) (Lim & Horwitz 1981, Masue et al. 1991). The conversion to 3-oxoalanine (C-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 GALNS cause mucopolysaccharidosis type IVA (MPSIVA, MIM:253000), also called Morquio A syndrome, a lysosomal storage disease characterized by intracellular accumulation of KS and CS (Fukuda et al. 1992).

Literature References
PubMed ID Title Journal Year
7213753 Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase

Lim, CT, Horwitz, AL

Biochim Biophys Acta 1981
1794986 N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics

Orii, T, Sukegawa, K, Hashimoto, T, Masue, M

J Biochem 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

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

Biochemistry 1998
1522213 Mucopolysaccharidosis type IVA. N-acetylgalactosamine-6-sulfate sulfatase exonic point mutations in classical Morquio and mild cases

Yamagishi, A, Hanyu, Y, Hori, T, Iwata, H, Tomatsu, S, Ogawa, T, Nakashima, Y, Sukegawa, K, Fukuda, S, Masue, M

J Clin Invest 1992
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
Catalyst Activity

N-acetylgalactosamine-6-sulfatase activity of GALNS oligomer [lysosomal lumen]

Orthologous Events
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