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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap

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Human metabolism

Reactome's human metabolism drawn as one network. Each molecule has one home node; widely shared molecules also appear as dashed copies in other territories, linked from their details. .

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Metabolite; its structure appears as you zoom in
Reaction, arrow toward the product
Cofactors and co-substrates are written beside the reaction when you zoom in
Dashed ring: a copy of a widely shared molecule or cofactor in another territory or pathway
Compartment transfer of the molecule it is linked to
Binding, release or complex event; its proteins are named in its details
Outline: reactions mostly in the mitochondrion

Arrows follow Reactome's curated reaction direction.

GALNS oligomer hydrolyses sulfate from Gal6S in keratan sulfate

H2O + Gal(S)-GlcNAc(S)-Gal-GlcNAc(S)-Gal → SO4(2-) + Gal-GlcNAc(S)-Gal-GlcNAc(S)-Gal

Reactome: R-HSA-1630304
Compartment: lysosomal lumen
Rhea: None recorded

Inputs

H2OCHEBI:15377, H2O, lysosomal lumen
Gal(S)-GlcNAc(S)-Gal-GlcNAc(S)-GalCHEBI:63846, C34H58N2O35S3, lysosomal lumen

Outputs

SO4(2-)CHEBI:16189, O4S, lysosomal lumen
Gal-GlcNAc(S)-Gal-GlcNAc(S)-GalCHEBI:63850, C34H58N2O31S2, lysosomal lumen

Catalysed by

N-acetylgalactosamine-6-sulfatase activity of GALNS dimer [lysosomal lumen]EC 3.1.6.4

Literature

Purification and properties of human N-acetylgalactosamine-6-sulfate sulfatase Biochim Biophys Acta, 1981
N-acetylgalactosamine-6-sulfate sulfatase in human placenta: purification and characteristics J Biochem, 1991
Crystal structure of a covalent intermediate of endogenous human arylsulfatase A J Inorg Biochem, 2003
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 Biochemistry, 1998
Mucopolysaccharidosis type IVA. N-acetylgalactosamine-6-sulfate sulfatase exonic point mutations in classical Morquio and mild cases J Clin Invest, 1992
The structure of human GALNS reveals the molecular basis for mucopolysaccharidosis IV A J Mol Biol, 2012