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Proteins/UNIPROT:P51688

SGSH

Protein·UNIPROT:P51688N-sulphoglucosamine sulphohydrolase

Open in the graph

Catalyzes a step in lysosomal heparan sulfate degradation..

Function from UniProtKB (P51688), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

Inferred

Inferred — not reported by a source. Evidence key

GenCC submitters' classifications

1 row

mucopolysaccharidosis type 3A has a germline basis in SGSHDiseaseGenCC HGNC:10818|MONDO:0009655Inferred only

Statements

55 statements

55 statements · 7 reference databases · Subject: SGSH · Sources: Bgee, Hetionet, Human Protein Atlas, Reactome, Rhea, UniProt, WikiPathways

Search this listOrder

SGSH covaries with ADAM12ProteinHetionet hetionet:covaries:6448-8038Inferred only
SGSH is expressed in adrenal cortexAnatomyBgee ENSG00000181523Inferred only
SGSH is expressed in adrenal glandAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in adrenal glandAnatomyBgee ENSG00000181523Inferred only
SGSH is expressed in breastAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in cerebellumAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in colonAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in duodenumAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in endometriumAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in epididymisAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in gallbladderAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in granulocyteCell typeBgee ENSG00000181523Inferred only

Show the remaining 43

SGSH is expressed in left adrenal glandAnatomyBgee ENSG00000181523Inferred only
SGSH is expressed in left adrenal gland cortexAnatomyBgee ENSG00000181523Inferred only
SGSH is expressed in liverAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in lungAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in myocardiumAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in pancreasAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in parathyroid glandAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in placentaAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in prostate glandAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in right adrenal glandAnatomyBgee ENSG00000181523Inferred only
SGSH is expressed in right adrenal gland cortexAnatomyBgee ENSG00000181523Inferred only
SGSH is expressed in seminal vesicleAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in Small intestineAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in spleenAnatomyBgee ENSG00000181523Inferred only
SGSH is expressed in spleenAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in stomachAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in stromal cell of endometriumCell typeBgee ENSG00000181523Inferred only
SGSH is expressed in testisAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in thyroid glandAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in urinary bladderAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH is expressed in zone of skinAnatomyHuman Protein Atlas ENSG00000181523Inferred only
SGSH interacts with FBXO6ProteinHetionet hetionet:interacts:26270-6448Inferred only
SGSH interacts with UBCProteinHetionet hetionet:interacts:6448-7316Inferred only
SGSH participates in DiseasePathwayReactome R-HSA-1643685Inferred only
SGSH participates in Diseases of carbohydrate metabolismPathwayReactome R-HSA-5663084Inferred only
SGSH participates in Diseases of metabolismPathwayReactome R-HSA-5668914Inferred only
SGSH participates in Glycosaminoglycan degradationPathwayWikiPathways WP4815Inferred only
SGSH participates in Glycosaminoglycan metabolismPathwayReactome R-HSA-1630316Inferred only
SGSH participates in Heparan sulfate/heparin (HS-GAG) metabolismPathwayReactome R-HSA-1638091Inferred only
SGSH participates in HS-GAG degradationPathwayReactome R-HSA-2024096Inferred only
SGSH participates in MetabolismPathwayReactome R-HSA-1430728Inferred only
SGSH participates in Metabolism of carbohydrates and carbohydrate derivativesPathwayReactome R-HSA-71387Inferred only
SGSH participates in MPS IIIA - Sanfilippo syndrome APathwayReactome R-HSA-2206307Inferred only
SGSH participates in MucopolysaccharidosesPathwayReactome R-HSA-2206281Inferred only
AFF1 increases the expression of SGSHProteinHetionet hetionet:regulates:4299-6448Inferred only
CTCF increases the expression of SGSHProteinHetionet hetionet:regulates:10664-6448Inferred only
MACF1 increases the expression of SGSHProteinHetionet hetionet:regulates:23499-6448Inferred only
P4HA2 increases the expression of SGSHProteinHetionet hetionet:regulates:8974-6448Inferred only
PPOX increases the expression of SGSHProteinHetionet hetionet:regulates:5498-6448Inferred only
SGSH requires the cofactor Ca(2+)CompoundUniProt P51688Inferred only
D-glucosamine is a substrate of SGSHCompoundRhea RHEA:17881Inferred only
N-sulfo-D-glucosamine is a substrate of SGSHCompoundRhea RHEA:17881Inferred only
sulfate is a substrate of SGSHCompoundRhea RHEA:17881Inferred only

Reactions

5 reactions

water+MAM02055lproton+MAM02066l+sulfate
Catalysed by SGSH
Reaction pageLeft to rightBalancedEC 3.10.1.1lysosome
Human-GEM MAR07227CC BY 4.0Reference data
water+MAM02076lproton+MAM02077l+sulfate
Catalysed by SGSH
Reaction pageLeft to rightBalancedEC 3.10.1.1lysosome

Other names

3 names

Resolves to: SGSH

Also called: HSSSulfoglucosamine sulfamidaseSulphamidase

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Human-GEM MAR07232

CC BY 4.0

Reference data

water+MAM02058lproton+MAM02059l+sulfate

Catalysed by SGSH

Reaction pageLeft to rightBalancedEC 3.10.1.1lysosome

Human-GEM MAR07238CC BY 4.0Reference data

water+MAM02064lproton+MAM02065l+sulfate

Catalysed by SGSH

Reaction pageLeft to rightBalancedEC 3.10.1.1lysosome

Human-GEM MAR07244CC BY 4.0Reference data

N-sulfo-D-glucosamine+waterD-glucosamine+sulfate

Catalysed by SGSH

Reaction pageDirection not assertedBalanced

Rhea RHEA:17881CC BY 4.0Reference data