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Aliases & Descriptions for IDS
(According to
1 HGNC ,
2 Entrez Gene ,
3 UniProtKB/Swiss-Prot ,
4 UniProtKB/TrEMBL , 5 OMIM , 6 GeneLoc
, and/or 7 Ensembl ,
8 miRBase )About This Section
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Aliases EC 3.1.6.13 3 Idursulfase 3 MPS2 2 , 5 OTTHUMP00000024207 2 OTTHUMP00000024210 2 S 5 SIDS 2 , 3 iduronate-2-sulfatase 2 idursulfase 2
Descriptions Alpha-L-iduronate sulfate sulfatase 2 , 3 iduronate 2-sulfatase 2 iduronate 2-sulfatase 14 kDa chain 2 iduronate 2-sulfatase 42 kDa chain 2
Search outside databases for aliases for IDS genePrevious GC identifers: GC0XM143088 GC0XM145395 GC0XM146459 GC0XM147269 GC0XM148266
Summaries for IDS (According to Entrez Gene ,
Wikipedia's
Gene Wiki ,
UniProtKB/Swiss-Prot ,
and/or
UniProtKB/TrEMBL )
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EntrezGene summary for IDS : Iduronate-2-sulfatase is required for the lysosomal degradation of heparan sulfate and dermatansulfate. Mutations in this X-chromosome gene that result in enzymatic deficiency lead to thesex-linked Mucopolysaccharidosis Type II, also known as Hunter Syndrome. Iduronate-2-sulfatase hasa strong sequence homology with human arylsulfatases A, B, and C, and humanglucosamine-6-sulfatase. A splice variant of this gene has been described. [provided by RefSeq] UniProtKB/Swiss-Prot: IDS_HUMAN, P22304 Function : Required for the lysosomal degradation of heparan sulfate and dermatan sulfate
Gene Wiki entry for IDS (Iduronate-2-sulfatase)
Genomic Location for IDS
(According to
GeneLoc and/or
HGNC , and/or
Entrez Gene (NCBI build 36) ,
and/or miRBase ,
Genomic Views according to
UCSC and
Ensembl ,
Transcription factor binding sites according to
SABiosciences )About This Section
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Genomic View : UCSC Golden Path with GeneCards custom track Transcription factor binding sites upstream to the IDS gene Entrez Gene cytogenetic band: Xq28 Ensembl cytogenetic band: Xq28 HGNC cytogenetic band: Xq27.3-q28 IDS Gene in genomic location: bands according to Ensembl, locations according to
(and/or Entrez Gene and/or Ensembl if different) GeneLoc gene densities for chromosome X GeneLoc Exon Structure
GeneLoc location for GC0XM148369:
(about GC identifiers )
Start:
148,368,206 bp from pter
End:
148,394,714 bp from pter
Size:
26,509 bases
Orientation:
minus strand
RefSeq DNA sequence: NC_000023.9 NT_011681.15 Proteins for IDS
(According to
1 UniProtKB ,
and/or Ensembl ,
Phosphorylation sites according to 2 Phosphosite ,
RefSeq according to NCBI ,
PDB rendering according to OCA and/or Proteopedia ,
Recombinant Proteins
from Invitrogen ,
Millipore ,
Sigma-Aldrich ,
R&D Systems ,
Enzo Life Sciences ,
Abnova ,
OriGene and/or,
Abcam ,
Biochemical Assays by
Invitrogen ,
Millipore ,
R&D Systems ,
Cell Signaling Technology , and/or
Enzo Life Sciences ,
Ontologies according to Gene
Ontology Consortium 01 Apr 2009 and
Entrez Gene ,
Antibodies by Invitrogen ,
Millipore ,
Sigma-Aldrich ,
R&D Systems ,
Cell Signaling Technology ,
Abcam ,
Abnova , and/or
Novus Biologicals )
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UniProtKB/Swiss-Prot: IDS_HUMAN, P22304 (See
protein sequence )Recommended Name: Iduronate 2-sulfatase precursor Size : 550 amino acids; 61873 Da
Cofactor : Binds 1 calcium ion per subunit (By similarity)
Subunit : Liver iduronate 2-sulfatase is composed of two major forms (A and B) which contain both a42 kDa and a 14 kDa polypeptides
Subcellular location : Lysosome
Secondary accessions : Q14604Alternative splicing : 2 isoforms : P22304-1 P22304-2
Post-translational modifications:
The conversion to 3-oxoalanine (also known as C-formylglycine, FGly), of a serine or cysteineresidue in prokaryotes and of a cysteine residue in eukaryotes, is critical for catalytic activity(By similarity)1
REFSEQ proteins (2 alternative transcripts):
NP_000193.1 NP_006114.1 ENSEMBL proteins: ENSP00000339801 ENSP00000359470 ENSP00000359472 ENSP00000359467 Human Recombinant Proteins Browse Origene for full length recombinant human proteins expressed in human HEK293 cells 1 Gene Ontology (GO) cellular component term (links to tree view) :
GO ID Qualified GO term Evidence PubMed IDs GO:0005764 lysosome
IEA --
About this table Antibodies for IDS: Assays for IDS:
Protein
Domains/ Families for IDS(According to InterPro , ProtoNet ,
UniProtKB , and/or BLOCKS ,
Sets of similar genes according to GeneDecks )
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Graphical View of Domain Structure for InterPro Entry P22304 ProtoNet protein and cluster: P22304
1 Blocks protein family : IPB000917 Sulfatase UniProtKB/Swiss-Prot: IDS_HUMAN, P22304 Similarity : Belongs to the sulfatase family
Gene Function for IDS
(According to MGI Jun 06 2009, UniProtKB ,
IUBMB ,and/or Genatlas ,
shRNA from
OriGene ,
Sigma-Aldrich , RNAi from
Sigma-Aldrich ,
RNAi Products ,
Clones , and
Q-PCR Products
from Invitrogen ,
Millipore ,
OriGene , and/or
Abnova ,
siRNAs from
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SYBR primers from OriGene ,
Cell-based Assays from Millipore ,
Ontologies according to Gene Ontology Consortium 01 Apr 2009 via
Entrez Gene .)
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               OriGene 29mer shRNA kit in GFP-retroviral vectors (see all 2 ): NM_000202 Applied Biosystems Silencer ® siRNAs for IDS Sigma-Aldrich siRNA and siRNA Panels for IDS Sigma-Aldrich shRNA for IDS Explore Sigma-Aldrich super-pooled esiRNAs                OriGene GFP tagged cDNA clones in CMV expression vector (see all 2 ): NM_000202                                  Myc/DDK tagged cDNA clones in CMV expression vector (see all 2 ): NM_000202                                  untagged cDNA clones in CMV expression vector (see all 4 ): AF050145  Primers: Browse
Quantitative PCR Central at Invitrogen for Q-PCR LUX™ Primers               OriGene genome-wide validated SYBR primer pairs: NM_000202 UniProtKB/Swiss-Prot: IDS_HUMAN, P22304 Function : Required for the lysosomal degradation of heparan sulfate and dermatan sulfateCatalytic activity : Hydrolysis of the 2-sulfate groups of the L-iduronate 2-sulfate units ofdermatan sulfate, heparan sulfate and heparin Enzyme Number (IUBMB): EC 3.1.6.13
Genatlas biochemistry entry for IDS :iduronate 2-sulfatase,lysosomal,acting on dermatan sulfate,heparan sulfate,catalyzing the firststep of degradation of glucosaminoglycans (mucopolysaccharides)
10 MGI mutant phenotypes (inferred from 1 allele ) (MGI details for Ids) :3 Gene Ontology (GO) molecular function terms (links to tree view) :
About this table
Pathways & Interactions for IDS
(Pathways according to Invitrogen
(maps by GeneGo ),
Millipore ,
Cell Signaling Technology ,
Sigma-Aldrich ,
KEGG
and/or UniProtKB ,
Sets of similar genes according to GeneDecks ,
Proteins Network according to
SABiosciences ,
Interactions according to 1 UniProtKB ,
2 MINT , and/or
3 STRING ,
with links to IntAct and
Ensembl ,
Ontologies according to Gene Ontology Consortium 01 Apr 2009 via
Entrez Gene .)
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1 Sigma-Aldrich "Your Favorite Gene" Pathway for IDS (Your Favorite Gene powered by Ingenuity) Gene Network CentralTM Interacting Genes and Proteins Network for IDS 4 Interacting proteins for IDS (ENSP00000339801 3 ) via UniProtKB, MINT, and/or STRING About this table 2 Gene Ontology (GO) biological process terms (links to tree view) :
GO ID Qualified GO term Evidence PubMed IDs GO:0008152 metabolic process
IEA -- GO:0030203 glycosaminoglycan metabolic process
IEA --
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Drugs & Compounds for IDS (Chemical Compounds according to UniProtKB , Enzo Life Sciences ,
Sigma-Aldrich , Tocris Bioscience , and/or
Novoseek and Drugs according to
Enzo Life Sciences and/or
PharmGKB )
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Browse Tocris compounds for IDS 9 Novoseek chemical compound relationships for IDS gene
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Transcripts for IDS(GenBank/EMBL/DDBJ Accessions according to
Unigene
(Build 219 Homo sapiens; Jun 2 2009) or GenBank , RefSeq according to Entrez Gene ,
DOTS (version 10), and/or
AceView ,
non coding RNAs according to
RNAdb ,
ESTs according to GeneTide ,
exon structure from GeneLoc ,
alternative splicing isoforms according to ASD and/or
ECgene ,
RNAi Products from Invitrogen ,
Millipore , and/or
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siRNAs from Applied Biosystems ,
Sigma-Aldrich ,
shRNA from
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OriGene ,
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               OriGene 29mer shRNA kit in GFP-retroviral vectors (see all 2 ): NM_000202 Sigma-Aldrich siRNA and siRNA Panels for IDS Sigma-Aldrich shRNA for IDS Explore Sigma-Aldrich super-pooled esiRNAs
Applied Biosystems Silencer ® siRNAs: NM_000202 NM_006123
REFSEQ mRNAs for IDS gene (2 alternative transcripts): NM_000202.4 NM_006123.3
Applied Biosystems TaqMan ® Gene Expression Assays: NM_000202 NM_006123
               OriGene GFP tagged cDNA clones in CMV expression vector (see all 2 ): NM_000202                                  Myc/DDK tagged cDNA clones in CMV expression vector (see all 2 ): NM_000202                                  untagged cDNA clones in CMV expression vector (see all 4 ): AF050145  
Additional cDNA sequence: AF050144.1 AF179280.1 AF179281.1 AK055600.1 AK057191.1 AK124660.1 AK294541.1 AK295815.1 AL049247.1 AL117536.1 BC006170.1 BX647357.1 CR625022.1 EF363095.1 L40586.1 M58342.1 U66044.1 U66045.1 U66055.1
24/45 DOTS entries (see all 45
): DT.448912 DT.95214772 DT.100812442 DT.95200577 DT.101957016 DT.100038327 DT.121299290 DT.75185698 DT.101961952 DT.121299157 DT.101977157 DT.97801656 DT.121643349 DT.91857589 DT.121643182 DT.86847177 DT.95326972 DT.121299130 DT.308816 DT.439576 DT.440153 DT.121299119 DT.121299190 DT.121640339
24/471 AceView cDNA sequences (see all 471
):BU625704 AL119592 CB155568 R87373 AA586715 AA460523 AA701678 AI419348 BF222034 AU138163 BU857935 BM968830 BC006170 AW612237 CF593282 BU182909 AA319199 BU538376 BE385802 BU955851 F05595 CB960647 U66052 CK821982
highest scoring ESTs for IDS :AF050145 L40586 AA010835 AA513794 AA776101 AA873599 AA993642 AI042325 AI089762 AI096538
Unigene Cluster for IDS: Iduronate 2-sulfatase Hs.460960 [show with all ESTs ] Unigene Representative Sequence: NM_000202 GeneLoc Exon Structure 5/8 Alternative Splicing Database (ASD) splice patterns (SP) for IDS (see all 8
) ExUns: 1 ^ 2 ^ 3a · 3b · 3c · 3d ^ 4a · 4b ^ 5 ^ 6a · 6b ^ 7a · 7b · 7c ^ 8a · 8b ^ 9a · 9b · 9c ^ 10 ^ 11a · 11b ^ 12a · 12b SP1 :             -   -     -   -                             SP2 :             -   -                                   SP3 :                   -   -                   -           SP4 :                   -   -                   -           SP5 :                                       -   -   -      
About this scheme ECgene alternative splicing isoforms for IDS 4 Ensembl transcripts including schematic representations : ENST00000340855
ENST00000370441
ENST00000370443
ENST00000370438
Expression for IDS
(Experimental results according to
1 GeneNote
and GNF BioGPS ,
probe sets-to-genes annotations according to
2 GeneAnnot ,
3 GeneTide ,
Sets of similar genes according to GeneDecks ,
Electronic Northern calculations according to data from
UniGene (Build 219 Homo sapiens),
SAGE tags according to
CGAP ,
plus additional links to
SOURCE , and/or
GNF
BioGPS , and/or
EXPOLDB , and/or
UniProtKB ,
Expression Assays from
Applied Biosystems
)
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IDS expression in normal and diseased human tissues Applied Biosystems TaqMan ® Gene Expression Assays for IDS 1 / 2 / 3
22 probe-sets matching IDS gene Data from
(Publications) and GNF BioGPS About these images About these images CGAP SAGE TAG: GTTCTCTTTGSOURCE GeneReport for Unigene cluster: Hs.460960 Expression variation in blood from EXPOLDB for IDS
UniProtKB/Swiss-Prot: IDS_HUMAN, P22304 Tissue specificity : Liver, kidney, lung, and placenta
Orthologs for IDS
(Orthologs according to
1,2 HomoloGene (2 older version, for species not in 1 newer version),
3 euGenes ,
4 SGD
and/or
5 MGI Jun 06 2009,
with possible further links to
Flybase
and/or
WormBase ,
Gene Trees according to Ensembl )
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Orthologs for IDS gene from 5/12 species (see all 12
)
About this table Species with no ortholog for IDS ENSEMBL Gene Tree for IDS Paralogs for IDS (Paralogs according to 1 HomoloGene and 2 Ensembl , Pseudogenes according to 3 Pseudogene.org )About This Section
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Paralogs for IDS gene ENSG00000176289 2
SNPs/Variants for IDS (According to the
1 NCBI SNP Database ,
2 Ensembl ,
3 PupaSUITE , and
UniProtKB ,
Linkage Disequilibrium by HapMap ,
Genotyping Reagents from
Applied Biosystems )
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HapMap Linkage Disequilibrium images for IDS (up to first 250kb)
Disorders & Mutations for IDS
(in which this Gene is Involved, According to
OMIM, UniProtKB ,
Novoseek , PharmGKB ,
Genatlas , GeneTests ,
Blood group antigen gene mutations by BGMUT ,
HGMD, GAD ,
HuGE Navigator ,
BCGD ,
and/or TGDB .)
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OMIM: 309900 UniProtKB/Swiss-Prot: IDS_HUMAN, P22304
Defects in IDS are the cause of mucopolysaccharidosis type 2 (MPS2) [MIM:309900]; alsoknown as Hunter syndrome. MPS2 is an X-linked lysosomal storage disease characterized byintracellular accumulation of heparan sulfate and dermatan sulfate and their excretion in urine.Most children with MPS2 have a severe form with early somatic abnormalities including skeletaldeformities, hepatosplenomegaly, and progressive cardiopulmonary deterioration. A prominentfeature is neurological damage that presents as developmental delay and hyperactivity butprogresses to mental retardation and dementia. They die before 15 years of age, usually as aresult of obstructive airway disease or cardiac failure. In contrast, those with a mild form ofMPS2 may survive into adulthood, with attenuated somatic complications and often without mentalretardation
8 Novoseek disease relationships for IDS gene
Disease
Score
Articles
PubMed IDs for Articles with Shared Sentences (# sentences)
hunters syndrome
98.91
203
17345556 (4), 1901826 (3), 8835216 (3), 18201153 (3) (see all 89 )
sudden infant death syndrome
80.38
19
2007878 (1), 8198848 (1), 15960133 (1), 8185361 (1) (see all 17 )
mucopolysaccharidosis
79.01
8
8103756 (1), 15146464 (1), 17284421 (1), 1303177 (1) (see all 6 )
lysosomal storage diseases
77.60
10
17217652 (1), 9337875 (1), 16480701 (1), 18331837 (1) (see all 8 )
asphyxia
24.24
3
12638728 (1), 1474151 (1)
mental retardation
23.79
1
17506108 (1)
leukemia
0.00
2
8835216 (1), 10808215 (1)
genetic disorder
0.00
2
1464193 (1)
About this table GeneTests: IDS Mucopolysaccharidosis Type II Human Gene Mutation Database : IDS
Medical News for IDS (Possibly Related Articles in
Doctor's Guide )
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Publications for IDS (in
PubMed .
Associations of this gene to articles via
1 Novoseek ,
2 HGNC ,
3 Entrez Gene ,
4 UniProtKB/Swiss-Prot ,
5 UniProtKB/TrEMBL ,
6 GAD , and/or
7 PharmGKB )
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10/176 PubMed articles for IDS gene (see all 176
): Hunter syndrome: isolation of an iduronate-2-sulfatase cDNA clone and analysis of patient DNA. (PubMed id 2122463) 1, 3, 4 Wilson P.J.... Hopwood J.J. (1990) Sequence of the human iduronate 2-sulfatase (IDS) gene. (PubMed id 8244397) 1, 3, 4 Wilson P.J.... Morris C.P. (1993) Identification of an alternative transcript from the human iduronate- 2-sulfatase (IDS) gene. (PubMed id 8530090) 1, 3, 4 Malmgren H.... Bondeson M.L. (1995) Mutations of the iduronate-2-sulfatase (IDS) gene in patients with Hunter syndrome (mucopolysaccharidosis II). (PubMed id 7981716) 1, 3, 4 Schroeder W.... Herrmann F.H. (1994) Molecular basis of iduronate-2-sulphatase gene mutations in patients with mucopolysaccharidosis type II (Hunter syndrome). (PubMed id 9950361) 1, 3, 4 Li P.... Thompson J.N. (1999) Mutational spectrum of the iduronate-2-sulfatase (IDS) gene in 36 unrelated Russian MPS II patients. (PubMed id 9921913) 1, 3, 4 Karsten S.... Bondeson M.-L. (1998) Molecular diagnosis of mucopolysaccharidosis type II (Hunter syndrome) by automated sequencing and computer-assisted interpretation: toward mutation mapping of the iduronate-2-sulfatase gene. (PubMed id 7887413) 1, 3, 4 Jonsson J.J.... Whitley C.B. (1995) The effect of four mutations on the expression of iduronate-2- sulfatase in mucopolysaccharidosis type II. (PubMed id 11731225) 1, 3, 4 Bonuccelli G....Filocamo M. (2001) Expression of five iduronate-2-sulfatase site-directed mutations. (PubMed id 10838181) 1, 3, 4 Villani G.R.D.... Di Natale P. (2000) Identification of 9 novel gene mutations in 19 unrelated Hunter syndrome (Mucopolysaccharidosis type II) patients. (PubMed id 10671065) 1, 3, 4 Karsten S.L.... Bondeson M.-L. (1998)
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Genome Databases showing IDS
(According to
Entrez Gene ,
HGNC ,
AceView ,
euGenes ,
Ensembl ,
miRBase ,
ECgene ,
and/or
H-InvDB )
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Other Databases showing IDS
(According to HUGE )
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--
Specialized Databases showing IDS (According to ATLAS , HORDE , IMGT , MTDB, LEIDEN , UniProtKB/Swiss-Prot , and/or UniProtKB/TrEMBL ,Wikipedia and/or GeneReviews via UniProtKB/Swiss-Prot )About This Section
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Name Description
GeneReviews http://www.genetests.org/query?gene=IDS
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-- Services for IDS (Reagents available from Applied Biosystems , Antibodies and assays by Cell
Signaling Technology , Abcam , Novus Biologicals ,Sigma-Aldrich , R&D Systems , Millipore , Abnova , and/or Invitrogen , Clones available from OriGene ,and/or Invitrogen , Drugs and/or compounds by Sigma-Aldrich , Enzo Life Sciences , and/or Tocris Bioscience )About This Section
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Products for IDS:
Search Tocris compounds for IDS
Antibodies for IDS
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GeneCards Homepage - Last full update: 2 Jul 2009
Incremental update: 13 Oct 2009