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Ids iduronate 2-sulfatase [ Mus musculus (house mouse) ]

Gene ID: 15931, updated on 2-Nov-2024

Summary

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Official Symbol
Idsprovided by MGI
Official Full Name
iduronate 2-sulfataseprovided by MGI
Primary source
MGI:MGI:96417
See related
Ensembl:ENSMUSG00000035847 AllianceGenome:MGI:96417
Gene type
protein coding
RefSeq status
VALIDATED
Organism
Mus musculus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Summary
Enables iduronate-2-sulfatase activity. Involved in dermatan sulfate catabolic process and heparan sulfate proteoglycan catabolic process. Located in lysosome. Is active in lysosomal lumen. Is expressed in nervous system and neural retina. Used to study mucopolysaccharidosis II. Human ortholog(s) of this gene implicated in mucopolysaccharidosis II. Orthologous to human IDS (iduronate 2-sulfatase). [provided by Alliance of Genome Resources, Nov 2024]
Expression
Broad expression in cortex adult (RPKM 56.7), frontal lobe adult (RPKM 54.0) and 20 other tissues See more
Orthologs
human all
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Genomic context

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See Ids in Genome Data Viewer
Location:
X A7.2; X 36.01 cM
Exon count:
19
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) X NC_000086.8 (69386675..69429437, complement)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) X NC_000086.7 (70343069..70385834, complement)

Chromosome X - NC_000086.8Genomic Context describing neighboring genes Neighboring gene AF4/FMR2 family, member 2 Neighboring gene ribosomal protein S6 pseudogene Neighboring gene RIKEN cDNA 1700111N16 gene Neighboring gene RIKEN cDNA 1700020N15 gene Neighboring gene STARR-seq mESC enhancer starr_47309 Neighboring gene STARR-seq mESC enhancer starr_47310 Neighboring gene STARR-seq mESC enhancer starr_47311 Neighboring gene STARR-seq mESC enhancer starr_47312 Neighboring gene STARR-seq mESC enhancer starr_47313 Neighboring gene RIKEN cDNA 4930567H17 gene Neighboring gene STARR-seq mESC enhancer starr_47315 Neighboring gene endothelium and lymphocyte associated ASCH domain 1

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: Mouse ENCODE transcriptome data Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

Bibliography

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Related articles in PubMed

  1. The characterization of a murine model of mucopolysaccharidosis II (Hunter syndrome). Garcia AR, et al. J Inherit Metab Dis, 2007 Nov. PMID 17876721
  2. CNS penetration of intrathecal-lumbar idursulfase in the monkey, dog and mouse: implications for neurological outcomes of lysosomal storage disorder. Calias P, et al. PLoS One, 2012. PMID 22279584, Free PMC Article
  3. Enzyme replacement therapy (ERT) procedure for mucopolysaccharidosis type II (MPS II) by intraventricular administration (IVA) in murine MPS II. Higuchi T, et al. Mol Genet Metab, 2012 Sep. PMID 22704483
  4. Auditory characteristics and therapeutic effects of enzyme replacement in mouse model of the mucopolysaccharidosis (MPS) II. Hong SH, et al. Am J Med Genet A, 2012 Sep. PMID 22847837
  5. Cloning and characterization of the cDNA for the murine iduronate sulfatase gene. Daniele A, et al. Genomics, 1993 Jun. PMID 8325651

See all (48) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Transferrin Receptor-Targeted Iduronate-2-sulfatase Penetrates the Blood-Retinal Barrier and Improves Retinopathy in Mucopolysaccharidosis II Mice.
    Title: Transferrin Receptor-Targeted Iduronate-2-sulfatase Penetrates the Blood-Retinal Barrier and Improves Retinopathy in Mucopolysaccharidosis II Mice.
  2. High-Throughput Liquid Chromatography-Tandem Mass Spectrometry Quantification of Glycosaminoglycans as Biomarkers of Mucopolysaccharidosis II.
    Title: High-Throughput Liquid Chromatography-Tandem Mass Spectrometry Quantification of Glycosaminoglycans as Biomarkers of Mucopolysaccharidosis II.
  3. We also show that mucopolysaccharidosis type II patient fibroblasts harboring different mutations spanning the IDS gene exhibit perturbed FGF signaling-related markers expression. Our work opens a new venue to discover possible druggable novel key targets in mucopolysaccharidosis type II
    Title: FGF signaling deregulation is associated with early developmental skeletal defects in animal models for mucopolysaccharidosis type II (MPSII).
  4. While the iduronate 2-sulfatase sp replacement results in increased enzyme secretion, the addition of the ApoB-BD allows efficient BBB transcytosis and restoration of sulphamidase activity in the brain of treated mice.
    Title: A highly secreted sulphamidase engineered to cross the blood-brain barrier corrects brain lesions of mice with mucopolysaccharidoses type IIIA.
  5. Ids crossing the blood-brain barrier corrects CNS defects in MPSII mice.
    Title: IDS crossing of the blood-brain barrier corrects CNS defects in MPSII mice.
  6. Iduronate-2-sulfatase (IDS) is localized in lysosomes in pancreatic islet cells and expression is regulated by glucose. IDS has a potential role in the normal pathway of lysosomal degradation of secretory peptides.
    Title: Identification of iduronate-2-sulfatase in mouse pancreatic islets.
Submit: New GeneRIF Correction

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics  (MGI)

Pathways from PubChem

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Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

Gene Ontology Provided by MGI

Function Evidence Code Pubs
enables calcium ion binding ISO
Inferred from Sequence Orthology
more info
  
enables calcium ion binding ISS
Inferred from Sequence or Structural Similarity
more info
  
enables iduronate-2-sulfatase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables iduronate-2-sulfatase activity IMP
Inferred from Mutant Phenotype
more info
 PubMed 
enables iduronate-2-sulfatase activity ISO
Inferred from Sequence Orthology
more info
  
enables iduronate-2-sulfatase activity ISS
Inferred from Sequence or Structural Similarity
more info
  
enables protein binding IPI
Inferred from Physical Interaction
more info
 PubMed 
enables sulfuric ester hydrolase activity IDA
Inferred from Direct Assay
more info
 PubMed 
Process Evidence Code Pubs
involved_in dermatan sulfate catabolic process IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in glycosaminoglycan catabolic process ISO
Inferred from Sequence Orthology
more info
  
involved_in glycosaminoglycan catabolic process ISS
Inferred from Sequence or Structural Similarity
more info
  
involved_in heparan sulfate proteoglycan catabolic process IMP
Inferred from Mutant Phenotype
more info
 PubMed 
Component Evidence Code Pubs
is_active_in cytoplasm IBA
Inferred from Biological aspect of Ancestor
more info
  
is_active_in lysosomal lumen IDA
Inferred from Direct Assay
more info
 PubMed 
located_in lysosome IDA
Inferred from Direct Assay
more info
 PubMed 
is_active_in lysosome IMP
Inferred from Mutant Phenotype
more info
 PubMed 
located_in lysosome ISO
Inferred from Sequence Orthology
more info
  

General protein information

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Preferred Names
iduronate 2-sulfatase
Names
alpha-L-iduronate sulfate sulfatase
iduronate sulfatase
NP_001388855.1
NP_001388856.1
NP_001388858.1
NP_001388859.1
NP_001388860.1
NP_001388861.1
NP_001388862.1
NP_001388863.1
NP_001388864.1
NP_001388865.1
NP_001388866.1
NP_034628.2
XP_006527909.1
XP_017173882.1
XP_017173883.1
XP_030107089.1
XP_030107090.1

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_001401926.1 → NP_001388855.1  iduronate 2-sulfatase isoform 2 precursor

    Status: VALIDATED

    Source sequence(s)
    AL672026, BX294168

  2. NM_001401927.1 → NP_001388856.1  iduronate 2-sulfatase isoform 2 precursor

    Status: VALIDATED

    Source sequence(s)
    BX294168

  3. NM_001401929.1 → NP_001388858.1  iduronate 2-sulfatase isoform 3 precursor

    Status: VALIDATED

    Source sequence(s)
    BX294168

  4. NM_001401930.1 → NP_001388859.1  iduronate 2-sulfatase isoform 4 precursor

    Status: VALIDATED

    Source sequence(s)
    BX294168

  5. NM_001401931.1 → NP_001388860.1  iduronate 2-sulfatase isoform 5

    Status: VALIDATED

    Source sequence(s)
    AL672026, BX294168

  6. NM_001401932.1 → NP_001388861.1  iduronate 2-sulfatase isoform 5

    Status: VALIDATED

    Source sequence(s)
    AL672026, BX294168

  7. NM_001401933.1 → NP_001388862.1  iduronate 2-sulfatase isoform 5

    Status: VALIDATED

    Source sequence(s)
    AL672026, BX294168

  8. NM_001401934.1 → NP_001388863.1  iduronate 2-sulfatase isoform 5

    Status: VALIDATED

    Source sequence(s)
    AL672026, BX294168

  9. NM_001401935.1 → NP_001388864.1  iduronate 2-sulfatase isoform 5

    Status: VALIDATED

    Source sequence(s)
    AL672026, BX294168

  10. NM_001401936.1 → NP_001388865.1  iduronate 2-sulfatase isoform 5

    Status: VALIDATED

    Source sequence(s)
    AL672026, BX294168

  11. NM_001401937.1 → NP_001388866.1  iduronate 2-sulfatase isoform 5

    Status: VALIDATED

    Source sequence(s)
    AL672026, BX294168

  12. NM_010498.4 → NP_034628.2  iduronate 2-sulfatase isoform 1 precursor

    See identical proteins and their annotated locations for NP_034628.2

    Status: VALIDATED

    Source sequence(s)
    BX294168
    Consensus CDS
    CCDS40996.1
    UniProtKB/Swiss-Prot
    Q08890, Q32KI7, Q3TM30
    UniProtKB/TrEMBL
    Q3V1R8
    Related
    ENSMUSP00000099046.3, ENSMUST00000101509.9
    Conserved Domains (2) summary
    COG3119
    Location:35 → 543
    AslA; Arylsulfatase A or related enzyme [Inorganic ion transport and metabolism]
    cd16030
    Location:40 → 543
    iduronate-2-sulfatase; iduronate-2-sulfatase

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

  1. NC_000086.8 Reference GRCm39 C57BL/6J

    Range
    69386675..69429437 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_017318394.2 → XP_017173883.1  iduronate 2-sulfatase isoform X2

    UniProtKB/TrEMBL
    Q684Q4
    Conserved Domains (1) summary
    cd16030
    Location:1 → 454
    iduronate-2-sulfatase

  2. XM_017318393.2 → XP_017173882.1  iduronate 2-sulfatase isoform X2

    UniProtKB/TrEMBL
    Q684Q4
    Conserved Domains (1) summary
    cd16030
    Location:1 → 454
    iduronate-2-sulfatase

  3. XM_030251230.1 → XP_030107090.1  iduronate 2-sulfatase isoform X2

    UniProtKB/TrEMBL
    Q684Q4
    Conserved Domains (1) summary
    cd16030
    Location:1 → 454
    iduronate-2-sulfatase

  4. XM_030251229.1 → XP_030107089.1  iduronate 2-sulfatase isoform X2

    UniProtKB/TrEMBL
    Q684Q4
    Conserved Domains (1) summary
    cd16030
    Location:1 → 454
    iduronate-2-sulfatase

  5. XM_006527846.5 → XP_006527909.1  iduronate 2-sulfatase isoform X3

    UniProtKB/TrEMBL
    Q684Q4
    Conserved Domains (1) summary
    cd16030
    Location:1 → 411
    iduronate-2-sulfatase

Suppressed Reference Sequence(s)

The following Reference Sequences have been suppressed. Explain

  1. NM_001038990.1: Suppressed sequence

    Description
    NM_001038990.1: This RefSeq was permanently suppressed because it was determined to be an artifact in sequencing due to the presence of microsatellites.

  2. NM_001038991.1: Suppressed sequence

    Description
    NM_001038991.1: This RefSeq was permanently suppressed because it was determined to be an artifact in sequencing due to the presence of microsatellites.
Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q08890.3 GenPept UniProtKB/Swiss-Prot:Q08890

Gene LinkOut

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