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iduronate 2-sulfatase

Summary

Gene Symbol

IDS

Aliases

Hunter syndrome
ID2S

Organism

Homo sapiens (human)

External Links

NCBI Gene

3423

HGNC

5389

KEGG Gene ID

hsa:3423

PubChem

3423

Alliance of Genome Resources

HGNC:5389

Annotation

Keyword

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

Proteins

Displaying **all 2** entries
UniProt	Protein Name
B4DGD7
P22304	Alpha-L-iduronate sulfate sulfatase

Annotation information from UniProt.

Gene Ontology (GO)

Displaying **all 3** entries
GO Term	Evidence Code	PMID
dermatan sulfate catabolic process	IEA
glycosaminoglycan catabolic process	IDA	28593992
heparan sulfate proteoglycan catabolic process	IEA

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

dermatan sulfate catabolic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate catabolic process

mucopolysaccharide metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate 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

dermatan sulfate 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]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

sulfur compound catabolic process [inference]

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

monosaccharide catabolic process [inference]

dermatan sulfate catabolic process

organic substance catabolic process [inference]

macromolecule catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

carbohydrate catabolic process [inference]

monosaccharide catabolic process [inference]

dermatan sulfate catabolic process

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

carbohydrate derivative catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate catabolic process

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

amino disaccharide catabolic process [inference]

dermatan sulfate catabolic process

organonitrogen compound catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

cellular metabolic process [inference]

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate catabolic process

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

sulfur compound metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate catabolic process

sulfur compound catabolic process [inference]

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

sulfur compound catabolic process [inference]

primary metabolic process [inference]

carbohydrate metabolic process [inference]

monosaccharide metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate catabolic process

monosaccharide catabolic process [inference]

dermatan sulfate catabolic process

carbohydrate catabolic process [inference]

monosaccharide catabolic process [inference]

dermatan sulfate 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

small molecule metabolic process [inference]

monosaccharide metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate catabolic process

monosaccharide catabolic process [inference]

dermatan sulfate catabolic process

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate catabolic process

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

small molecule catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

monosaccharide catabolic process [inference]

dermatan sulfate catabolic process

organic substance metabolic process [inference]

carbohydrate metabolic process [inference]

monosaccharide metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate catabolic process

monosaccharide catabolic process [inference]

dermatan sulfate catabolic process

carbohydrate catabolic process [inference]

monosaccharide catabolic process [inference]

dermatan sulfate catabolic process

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate catabolic process

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

macromolecule metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate catabolic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate catabolic process

mucopolysaccharide metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate catabolic process

macromolecule catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate 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

dermatan sulfate catabolic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate catabolic process

mucopolysaccharide metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate 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

amino disaccharide metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate catabolic process

amino disaccharide catabolic process [inference]

dermatan sulfate catabolic process

carbohydrate derivative catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate catabolic process

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

amino disaccharide catabolic process [inference]

dermatan sulfate catabolic process

organonitrogen compound metabolic process [inference]

aminoglycan metabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate catabolic process

glycosaminoglycan metabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate catabolic process

mucopolysaccharide metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate 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

dermatan sulfate 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

dermatan sulfate catabolic process

protein catabolic process [inference]

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

carbohydrate catabolic process [inference]

monosaccharide catabolic process [inference]

dermatan sulfate catabolic process

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

carbohydrate derivative catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate catabolic process

glycoprotein catabolic process [inference]

proteoglycan catabolic process [inference]

heparan sulfate proteoglycan catabolic process

amino disaccharide catabolic process [inference]

dermatan sulfate catabolic process

organonitrogen compound catabolic process [inference]

aminoglycan catabolic process [inference]

glycosaminoglycan catabolic process

dermatan sulfate 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]

organic acid metabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

oxoacid metabolic process [inference]

carboxylic acid metabolic process [inference]

monocarboxylic acid metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate catabolic process

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

sulfur compound metabolic process [inference]

dermatan sulfate metabolic process [inference]

dermatan sulfate catabolic process

sulfur compound catabolic process [inference]

cellular catabolic process [inference]

organic acid catabolic process [inference]

carboxylic acid catabolic process [inference]

monocarboxylic acid catabolic process [inference]

dermatan sulfate catabolic process

sulfur compound catabolic process [inference]

Displaying **all 3** entries
GO Term	Evidence Code	PMID
cytoplasm	IBA
lysosomal lumen
lysosome	IDA	10838181

GO Hierarchy

cellular component

cellular anatomical entity [inference]

cytoplasm

membrane-enclosed lumen [inference]

organelle lumen [inference]

intracellular organelle lumen [inference]

vacuolar lumen [inference]

lysosomal lumen

organelle [inference]

membrane-bounded organelle [inference]

intracellular membrane-bounded organelle [inference]

vacuole [inference]

lytic vacuole [inference]

lysosome

intracellular organelle [inference]

intracellular membrane-bounded organelle [inference]

vacuole [inference]

lytic vacuole [inference]

lysosome

Displaying **all 3** entries
GO Term	Evidence Code	PMID
iduronate-2-sulfatase activity	IBA IDA	10838181
calcium ion binding	IDA	28593992
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 [inference]

iduronate-2-sulfatase activity

binding [inference]

protein binding

ion binding [inference]

cation binding [inference]

metal ion binding [inference]

calcium ion binding

Gene Ontology information from NCBI Gene and UniProt.

Human Protein Atlas

ENSG00000010404

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

K01136

Name

iduronate 2-sulfatase [EC:3.1.6.13]

References

15149955

Reactions

Displaying 1 entry
KEGG Reaction	Enzyme	Acceptor	Product
R07821	3.1.6.13	G15398PM	G91199CQ

Disease

Disease Ontology

Displaying entries **1 - 10** of **129** in total

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DO ID	Disease Name	Source
DOID:0050451	Brugada syndrome	DisGeNET
DOID:0050632	oculocutaneous albinism	DisGeNET
DOID:0050635	alternating hemiplegia of childhood	DisGeNET
DOID:0050759	myotonic dystrophy type 2	DisGeNET
DOID:0050811	congenital adrenal hyperplasia	DisGeNET
DOID:0050847	sleep apnea	DisGeNET
DOID:0050848	obstructive sleep apnea	DisGeNET DisGeNET
DOID:0060041	autism spectrum disorder	DisGeNET
DOID:0060215	Balo concentric sclerosis	DisGeNET
DOID:0060260	ptosis	DisGeNET

The Human Phenotype Ontology

Displaying entries **1 - 10** of **109** in total

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HPO ID	HPO Term
HP:0000023	Inguinal hernia
HP:0000158	Macroglossia
HP:0000179	Thick lower lip vermilion
HP:0000212	Gingival overgrowth
HP:0000238	Hydrocephalus
HP:0000256	Macrocephaly
HP:0000268	Dolichocephaly
HP:0000280	Coarse facial features
HP:0000293	Full cheeks
HP:0000336	Prominent supraorbital ridges

Displaying **all 3** entries
Disease ID	Disease Name
ORPHA:217093	mucopolysaccharidosis type 2, attenuated form
ORPHA:217085	mucopolysaccharidosis type 2, severe form
OMIM:309900	mucopolysaccharidosis type 2

PubChem Disease

GHR Health Conditions

Mucopolysaccharidosis type II

MedGen Diseases

Mucopolysaccharidosis, MPS-II

OMIM Phenotypes

MUCOPOLYSACCHARIDOSIS, TYPE II; MPS2

KEGG Diseases

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

Ortholog

Displaying entries **1 - 10** of 73 in total

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Species	Gene ID	Alliance of Genome Resources	Orthologous MAtrix
Drosophila melanogaster	38423	FB:FBgn0035445
Callorhinchus milii	103178266		CALMI01816
Latimeria chalumnae	102348043		LATCH16446
Danio rerio	559959	ZFIN:ZDB-GENE-061215-37	DANRE06937
Astyanax mexicanus	103032782		ASTMX15720
Ictalurus punctatus	108268807		ICTPU34536
Salmo salar	106611990		SALSA44838
Salmo trutta	115205471		SALTR111546
Gadus morhua	115552660		GADMO02773
Oreochromis niloticus	100701301		ORENI46251

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Last updated: August 19, 2024