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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. .

Territories

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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.

SGSH hydrolyses Heparan sulfate chain(2)

2 H2O + GlcNS6S-IdoAS2-GlcNS3S- group → 2 SO4(2-) + GlcN-IdoA2S-GlcNS3S- group

Reactome: R-HSA-1678708
Compartment: lysosomal lumen
Rhea: None recorded

Inputs

2 H2OCHEBI:15377, H2O, lysosomal lumen
GlcNS6S-IdoAS2-GlcNS3S- groupCHEBI:63805, C41H67N2O49S5, lysosomal lumen

Outputs

2 SO4(2-)CHEBI:16189, O4S, lysosomal lumen
GlcN-IdoA2S-GlcNS3S- groupCHEBI:63806, C41H67N2O43S3, lysosomal lumen

Catalysed by

N-sulfoglucosamine sulfohydrolase activity of SGSH:Ca2+ [lysosomal lumen]EC 3.10.1.1

Literature

Cloning of the sulphamidase gene and identification of mutations in Sanfilippo A syndrome Nat Genet, 1995
Novel mutations in Sanfilippo A syndrome: implications for enzyme function Hum Mol Genet, 1997