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

Summary

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

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_000086.8 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: 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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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

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.
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.
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).
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.
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.
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)

Endonuclease-mediated (4) 1 citation
Targeted (3) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

AW214631 (e-PCR)
- UniSTS:181292

IDS (e-PCR)
- UniSTS:266923

PMC311008P2 (e-PCR)
- UniSTS:266923

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

EC 3.1.6.13

NP_001388856.1

EC 3.1.6.13

NP_001388858.1

EC 3.1.6.13

NP_001388859.1

EC 3.1.6.13

NP_001388860.1

EC 3.1.6.13

NP_001388861.1

EC 3.1.6.13

NP_001388862.1

EC 3.1.6.13

NP_001388863.1

EC 3.1.6.13

NP_001388864.1

EC 3.1.6.13

NP_001388865.1

EC 3.1.6.13

NP_001388866.1

EC 3.1.6.13

NP_034628.2

EC 3.1.6.13

XP_006527909.1

EC 3.1.6.13

XP_017173882.1

EC 3.1.6.13

XP_017173883.1

EC 3.1.6.13

XP_030107089.1

EC 3.1.6.13

XP_030107090.1

EC 3.1.6.13

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)

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

Status: VALIDATED

Source sequence(s)

AL672026, BX294168
NM_001401927.1 → NP_001388856.1 iduronate 2-sulfatase isoform 2 precursor

Status: VALIDATED

Source sequence(s)

BX294168
NM_001401929.1 → NP_001388858.1 iduronate 2-sulfatase isoform 3 precursor

Status: VALIDATED

Source sequence(s)

BX294168
NM_001401930.1 → NP_001388859.1 iduronate 2-sulfatase isoform 4 precursor

Status: VALIDATED

Source sequence(s)

BX294168
NM_001401931.1 → NP_001388860.1 iduronate 2-sulfatase isoform 5

Status: VALIDATED

Source sequence(s)

AL672026, BX294168
NM_001401932.1 → NP_001388861.1 iduronate 2-sulfatase isoform 5

Status: VALIDATED

Source sequence(s)

AL672026, BX294168
NM_001401933.1 → NP_001388862.1 iduronate 2-sulfatase isoform 5

Status: VALIDATED

Source sequence(s)

AL672026, BX294168
NM_001401934.1 → NP_001388863.1 iduronate 2-sulfatase isoform 5

Status: VALIDATED

Source sequence(s)

AL672026, BX294168
NM_001401935.1 → NP_001388864.1 iduronate 2-sulfatase isoform 5

Status: VALIDATED

Source sequence(s)

AL672026, BX294168
NM_001401936.1 → NP_001388865.1 iduronate 2-sulfatase isoform 5

Status: VALIDATED

Source sequence(s)

AL672026, BX294168
NM_001401937.1 → NP_001388866.1 iduronate 2-sulfatase isoform 5

Status: VALIDATED

Source sequence(s)

AL672026, BX294168
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

NC_000086.8 Reference GRCm39 C57BL/6J

Range

69386675..69429437 complement

Download

GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

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
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
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
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
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

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.
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.