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N-sulfoglucosamine sulfohydrolase

Summary

Gene Symbol

SGSH

Aliases

HSS
MPS3A
SFMD
mucopolysaccharidosis type IIIA
sulfamidase

Organism

Homo sapiens (human)

External Links

NCBI Gene

6448

HGNC

10818

KEGG Gene ID

hsa:6448

PubChem

6448

Alliance of Genome Resources

HGNC:10818

Annotation

Keyword

3D-structure
Calcium
Direct protein sequencing
Disease variant
Disulfide bond
Glycoprotein
Hydrolase
Lysosome
Metal-binding
Mucopolysaccharidosis
Reference proteome
Signal

Proteins

Displaying 1 entry
UniProt	Protein Name
P51688	Sulfoglucosamine sulfamidase Sulphamidase

Annotation information from UniProt.

Gene Ontology (GO)

Displaying **all 4** entries
GO Term	Evidence Code	PMID
glycosaminoglycan catabolic process	IBA IDA	15146460 7493035
determination of adult lifespan	IEA
motor behavior	IEA
heparan sulfate proteoglycan catabolic process	IBA IMP TAS	15146460

GO Hierarchy

biological process

metabolic process [inference]

nitrogen compound metabolic process [inference]

organonitrogen compound metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan catabolic process

protein metabolic process [inference]

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

organonitrogen compound catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

catabolic process [inference]

cellular catabolic process [inference]

sulfur compound catabolic process [inference]

heparan sulfate proteoglycan catabolic process

organic substance catabolic process [inference]

macromolecule catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

carbohydrate derivative catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

organonitrogen compound catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

cellular metabolic process [inference]

sulfur compound metabolic process [inference]

sulfur compound catabolic process [inference]

heparan sulfate proteoglycan catabolic process

cellular catabolic process [inference]

sulfur compound catabolic process [inference]

heparan sulfate proteoglycan catabolic process

primary metabolic process [inference]

protein metabolic process [inference]

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

organic substance metabolic process [inference]

macromolecule metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan catabolic process

macromolecule catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

protein metabolic process [inference]

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

carbohydrate derivative metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan catabolic process

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

carbohydrate derivative catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

organonitrogen compound metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan catabolic process

protein metabolic process [inference]

glycoprotein metabolic process [inference]

proteoglycan metabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

organonitrogen compound catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

organic substance catabolic process [inference]

macromolecule catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

carbohydrate derivative catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

organonitrogen compound catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

cellular process [inference]

cellular metabolic process [inference]

sulfur compound metabolic process [inference]

sulfur compound catabolic process [inference]

heparan sulfate proteoglycan catabolic process

cellular catabolic process [inference]

sulfur compound catabolic process [inference]

heparan sulfate proteoglycan catabolic process

multicellular organismal process [inference]

behavior [inference]

motor behavior

determination of adult lifespan

Displaying **all 3** entries
GO Term	Evidence Code	PMID
lysosomal lumen
extracellular exosome	HDA	23533145
lysosome	IDA	15146460

GO Hierarchy

cellular component

cellular anatomical entity [inference]

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

vacuolar lumen [inference]

lysosomal lumen

organelle [inference]

membrane-bounded organelle [inference]

vesicle [inference]

extracellular vesicle [inference]

extracellular exosome

intracellular membrane-bounded organelle [inference]

vacuole [inference]

lytic vacuole [inference]

lysosome

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

vacuole [inference]

lytic vacuole [inference]

lysosome

extracellular organelle [inference]

extracellular membrane-bounded organelle [inference]

extracellular vesicle [inference]

extracellular exosome

Displaying **all 4** entries
GO Term	Evidence Code	PMID
sulfuric ester hydrolase activity	IEA
N-sulfoglucosamine sulfohydrolase activity	IBA IDA TAS	15146460 7493035
metal ion binding	IEA
protein binding	IPI	15962010

GO Hierarchy

molecular function

catalytic activity [inference]

hydrolase activity [inference]

hydrolase activity, acting on ester bonds [inference]

sulfuric ester hydrolase activity

hydrolase activity, acting on acid sulfur-nitrogen bonds [inference]

N-sulfoglucosamine sulfohydrolase activity

binding [inference]

protein binding

ion binding [inference]

cation binding [inference]

metal ion binding

Gene Ontology information from NCBI Gene and UniProt.

Human Protein Atlas

ENSG00000181523

Tissue with high expression from Human Protein Atlas. Tissues that are highly expressed are highlighted.

Data available from v23.0.proteinatlas.org. Image credit: Human Protein Atlas

KEGG BRITE Database

Orthology

K01565

Name

N-sulfoglucosamine sulfohydrolase [EC:3.10.1.1]

References

7493035

Rhea

RHEA:17881

H₂O + N-sulfo-D-glucosamine

sulfate + D-glucosamine

Enzyme

3.10.1.1

PMID

6849981

Disease

Disease Ontology

Displaying entries **1 - 10** of 80 in total

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DO ID	Disease Name	Source
DOID:0050426	Stevens-Johnson syndrome	DisGeNET
DOID:0050441	mucosulfatidosis	DisGeNET
DOID:0050572	cone-rod dystrophy	DisGeNET
DOID:0050700	cardiomyopathy	DisGeNET
DOID:0060215	Balo concentric sclerosis	DisGeNET
DOID:0080348	Alzheimer's disease 1	DisGeNET
DOID:0090005	Schwartz-Jampel syndrome 1	DisGeNET
DOID:0110035	Alzheimer's disease 2	DisGeNET
DOID:0110037	Alzheimer's disease 5	DisGeNET
DOID:0110038	Alzheimer's disease 6	DisGeNET

The Human Phenotype Ontology

Displaying entries **1 - 10** of 27 in total

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HPO ID	HPO Term
HP:0000007	Autosomal recessive inheritance
HP:0000023	Inguinal hernia
HP:0000250	Dense calvaria
HP:0000280	Coarse facial features
HP:0000365	Hearing impairment
HP:0000664	Synophrys
HP:0000752	Hyperactivity
HP:0000900	Thickened ribs
HP:0000943	Dysostosis multiplex
HP:0001007	Hirsutism

Displaying 1 entry
Disease ID	Disease Name
OMIM:252900	mucopolysaccharidosis type 3A

PubChem Disease

GHR Health Conditions

Mucopolysaccharidosis type III

MedGen Diseases

Mucopolysaccharidosis, MPS-III-A

OMIM Phenotypes

MUCOPOLYSACCHARIDOSIS, TYPE IIIA; MPS3A

KEGG Diseases

Gene-Disease-Co-Occurrences-in-Literature (PubChem)

Ortholog

Displaying entries **1 - 10** of 68 in total

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Drosophila melanogaster	42266	FB:FBgn0038660
Ciona intestinalis	113474074		CIOIN05530
Latimeria chalumnae	102349697		LATCH18346
Danio rerio	563849	ZFIN:ZDB-GENE-030131-4958	DANRE22917
Ictalurus punctatus	108259505		ICTPU22321
Electrophorus electricus	113585727		ELEEL34790
Salmo trutta	115147563		SALTR35145
Salmo trutta	115181670		SALTR35145
Oreochromis niloticus	100699960		ORENI63789
Sparus aurata	115589512		SPAAU54202

GlyTouCan

GlyComb

GlycoPOST

UniCarb-DR

All Resources

Other Information

Tools

Search Tools

Summary

External Links

Annotation

KEGG BRITE Database

PubChem Disease

International Collaboration

Acknowledgements