GNS:Ca2+ cleaves sulfate from GlcNAc-6-sulfate units of keratan sulfate
H2O + GlcNAc(S)-Gal-GlcNAc(S)-Gal → SO4(2-) + GlcNAc-Gal-GlcNAc(S)-Gal
- Reactome
- R-HSA-1638032
- Compartment
- lysosomal lumen
- Rhea
- None recorded
Inputs
- H2OCHEBI:15377, H2O, lysosomal lumen
- GlcNAc(S)-Gal-GlcNAc(S)-GalCHEBI:63851, C28H48N2O27S2, lysosomal lumen
Outputs
- SO4(2-)CHEBI:16189, O4S, lysosomal lumen
- GlcNAc-Gal-GlcNAc(S)-GalCHEBI:63852, C28H48N2O24S, lysosomal lumen
Catalysed by
- N-acetylglucosamine-6-sulfatase activity of GNS:Ca2+ [lysosomal lumen]EC 3.1.6.14
Literature
- A cDNA clone for human glucosamine-6-sulphatase reveals differences between arylsulphatases and non-arylsulphatases Biochem J, 1992
- Human glucosamine-6-sulfatase cDNA reveals homology with steroid sulfatase Biochem Biophys Res Commun, 1988
- Mucopolysaccharidosis type IIID: 12 new patients and 15 novel mutations Hum Mutat, 2010