Affinage

IDS

Iduronate 2-sulfatase · UniProt P22304

Length
550 aa
Mass
61.9 kDa
Annotated
2026-06-10
100 papers in source corpus 21 papers cited in narrative 21 extracted findings
Cross-family judge vs UniProt: Affinage preferred faithfulness: 7/7 claims corpus-supported (100%)

Mechanistic narrative

Synthesis pass · prose summary of the discoveries below

IDS encodes iduronate-2-sulfatase, a lysosomal sulfatase whose deficiency causes the X-linked lysosomal storage disorder mucopolysaccharidosis type II (Hunter syndrome) (PMID:19679226, PMID:28543354). The gene spans ~24 kb across nine exons and is driven by a TATA-less, GC-box-containing promoter characteristic of housekeeping genes (PMID:8244397). The enzyme is synthesized as 76- and 90-kDa precursors that are post-translationally processed through intermediate forms to mature 55- and 45-kDa polypeptides; because precursors are poorly secreted, IDS transfers to enzyme-deficient cells preferentially by cell-to-cell contact, after which the transferred polypeptides are correctly matured in recipient cells (PMID:9024795). Pathogenic missense variants typically retain only precursor protein with impaired or absent maturation and reduced catalytic activity, and total loss of IDS produces the neuronopathic phenotype while low residual activity can spare the CNS (PMID:28543354, PMID:30639582, PMID:31877959). A major share of disease-causing alleles arise from genomic rearrangement: non-allelic homologous recombination between IDS and the adjacent IDSP1 (IDS-2) pseudogene ~80–90 kb telomeric on Xq28—whose exon 3 sequence is 100% identical to IDS—drives recurrent inversions and intragenic deletions at intron 7/exon 3 hotspots (PMID:7633410, PMID:8528670, PMID:8807335), while additional rearrangements occur through illegitimate recombination at short direct-repeat hotspots (PMID:9244428, PMID:21593745). A second large class of mutations, including synonymous variants, disrupts splicing by activating cryptic splice sites and engaging nonsense-mediated and nonstop mRNA decay (PMID:16699754, PMID:17063374, PMID:21829674). The capacity of circulating IDS to cross the blood-brain barrier and correct CNS glycosaminoglycan accumulation has been exploited therapeutically in mouse models (PMID:19679226).

Mechanistic history

Synthesis pass · year-by-year structured walk · 13 steps
  1. 1993 High

    Establishing the genomic architecture and promoter character of IDS defined the gene as a constitutively expressed locus and provided the structural framework for all subsequent mutation analysis.

    Evidence Genomic cloning, sequencing of nine exons, and promoter analysis

    PMID:8244397

    Open questions at the time
    • Does not address protein structure or catalytic mechanism
    • No functional confirmation of housekeeping expression in vivo
  2. 1992 Medium

    Detection of large deletions and rearrangements in ~20% of MPS II patients revealed that a region of IDS is structurally unstable, pointing to a recombination hotspot before its mechanism was known.

    Evidence Southern blot of 46 unrelated MPS-II patients with full-length cDNA probe and breakpoint mapping

    PMID:1303177

    Open questions at the time
    • Did not identify the recombining partner sequence
    • Breakpoints not sequenced at nucleotide resolution
  3. 1995 High

    Identification of the IDSP1/IDS-2 pseudogene and recombination junctions explained the structural instability as non-allelic homologous recombination, accounting for a recurrent (~13%) cause of Hunter syndrome and a confound for genomic diagnostics.

    Evidence Southern blot and junction sequencing of inversion alleles; mapping of homologous pseudogene sequences on Xq28

    PMID:7633410 PMID:8528670

    Open questions at the time
    • Frequency estimates depend on cohort and detection method
    • Mechanism of inversion formation not reconstituted experimentally
  4. 1996 Medium

    Sequencing of an intragenic exon IV–VII deletion junction confirmed that gene-pseudogene exchange produces not only inversions but also deletions, extending the homologous-recombination model to multiple rearrangement classes.

    Evidence Southern blot, PCR, and breakpoint sequencing in two patients

    PMID:8807335

    Open questions at the time
    • Based on two patients
    • Recombination not experimentally reconstituted
  5. 1997 Medium

    Discovery of a recurrent heptamer at deletion junctions showed that, alongside homologous recombination, illegitimate recombination at short direct repeats contributes to IDS rearrangements.

    Evidence Southern blot, PCR, and junction sequencing of two deletions 30 kb apart

    PMID:9244428

    Open questions at the time
    • Single case study
    • Hotspot role of heptamer not proven mechanistically
  6. 1997 Medium

    Characterizing IDS biosynthesis defined the precursor-to-mature processing pathway and showed that intercellular enzyme transfer occurs chiefly by cell-to-cell contact rather than secretion, framing how cross-correction operates.

    Evidence Western blotting of processing in multiple cell lines plus coculture versus conditioned-medium transfer assays

    PMID:9024795

    Open questions at the time
    • Molecular basis of contact-dependent transfer not defined
    • Processing protease(s) not identified
  7. 1995 Medium

    Identification of an alternative transcript lacking the C-terminal domain raised the possibility of an additional IDS protein form.

    Evidence cDNA cloning and sequencing of a 1.4-kb alternative transcript

    PMID:8530090

    Open questions at the time
    • Functional significance of the truncated form not established
    • Protein product not detected in vivo
  8. 2006 Medium

    Systematic transcript analyses established that point and silent mutations frequently act by activating cryptic splice sites and that aberrant transcripts are subject to NMD, making splicing a dominant pathogenic mechanism distinct from coding changes.

    Evidence RT-PCR transcript cloning, real-time RT-PCR quantification, and cDNA/gDNA comparison across patient cohorts

    PMID:16495038 PMID:16699754 PMID:17063374

    Open questions at the time
    • Variable NMD susceptibility across alleles not fully explained
    • Splice factor mechanisms not defined in these studies
  9. 2009 High

    Systemic AAV delivery showed that circulating IDS crosses the blood-brain barrier to correct CNS glycosaminoglycan storage, establishing the therapeutic principle that peripheral enzyme can treat CNS disease.

    Evidence Systemic AAV2/5-hIDS in MPSII mouse pups with long-term biochemical/histological assessment distinguishing brain transduction from circulating enzyme

    PMID:19679226

    Open questions at the time
    • Molecular route of BBB crossing not identified
    • Mouse-to-human translation not addressed
  10. 2010 Medium

    Detection of wild-type IDS mRNA and protein in patients carrying only mutant genomic alleles raised the possibility of in vivo RNA-level correction of pathogenic mutations.

    Evidence Multiple orthogonal methods (pyrosequencing, SNuPE, real-time PCR, Western blot) with segregation analysis in three patients

    PMID:20104590

    Open questions at the time
    • Proposed RNA editing mechanism not directly proven
    • Limited to three patients
  11. 2019 Medium

    Integrating structural modeling with expression assays linked specific missense substitutions to helix deformation, impaired maturation, and loss of activity, and connected genotype severity to residual activity and CNS involvement.

    Evidence Homology modeling, enzyme activity assays, and immunoblotting of intracellular processing for numerous variants across cohorts

    PMID:27246110 PMID:28543354 PMID:30639582 PMID:31877959

    Open questions at the time
    • No experimental crystal structure
    • Quantitative activity thresholds protecting the CNS not precisely defined
  12. 2019 Medium

    Brain-targeted nanoparticle delivery of IDS restored enzyme activity and reduced storage and neuroinflammation in patient cells and MPSII mice, advancing CNS-directed enzyme replacement strategies.

    Evidence In vitro activity assays in patient fibroblasts and in vivo biochemical/histological assessment with g7-PLGA nanoparticles

    PMID:31022913

    Open questions at the time
    • Single lab
    • Durability and human translation not established
  13. 2015 Medium

    Splicing-factor and antisense experiments began to define the cis- and trans-acting regulation of IDS exon 3 and exon 8 splice sites relevant to therapeutic splice modulation.

    Evidence Minigene splicing assays, SRSF2/hnRNP E1 overexpression, and antisense oligonucleotide transfection in fibroblasts

    PMID:26407519

    Open questions at the time
    • Splice factor roles described as tentative
    • Antisense oligonucleotides generated additional aberrant products rather than correction

Open questions

Synthesis pass · forward-looking unresolved questions
  • The catalytic mechanism and high-resolution structure of human IDS, and the molecular basis of contact-dependent intercellular enzyme transfer, remain undefined in this corpus.
  • No experimentally determined IDS structure in the timeline
  • Mechanism of cell-to-cell IDS transfer unresolved
  • Sulfatase catalytic chemistry not directly characterized here

Mechanism profile

Synthesis pass · controlled-vocabulary classification · explore literature graph →
Molecular activity
GO:0016787 hydrolase activity 5 GO:0140098 catalytic activity, acting on RNA 2
Localization
GO:0005764 lysosome 1
Pathway
R-HSA-1430728 Metabolism 3 R-HSA-1643685 Disease 3

Evidence

Reading pass · 21 per-paper findings extracted from the source corpus
Year Finding Method Journal Conf PMIDs
1993 The IDS gene encoding iduronate-2-sulfatase spans approximately 24 kb and is split into nine exons. The promoter lacks a TATA box but contains GC box consensus sequences, consistent with housekeeping gene regulation. Genomic cloning and sequencing of overlapping genomic clones; promoter region analysis Genomics High 8244397
1995 Inversion of the IDS gene resulting from recombination between homologous sequences in intron 7 of IDS and sequences near exon 3 of the IDS-related locus (IDS-2/IDSP1), located within 90 kb telomeric of IDS, is a common cause (~13%) of Hunter syndrome. No deletions or insertions accompany the recombination. Southern blot analysis and nucleotide sequencing of rearrangement junctions in Hunter syndrome patients Human molecular genetics High 7633410
1995 An IDS-related locus (IDS-2/IDSP1) exists within 80 kb telomeric of the functional IDS gene on Xq28, containing sequences homologous to exons 2 and 3 and introns 2, 3, and 7 of IDS (exon 3 sequences show 100% identity). This locus complicates mutational analysis of genomic DNA from Hunter syndrome patients. Southern blot analysis, PCR amplification, and sequencing to map homologous sequences European journal of human genetics High 8528670
1992 Structural alterations (large deletions and major rearrangements) in the IDS gene were found in ~20% of MPS-II patients. A region within IDS is prone to structural alterations, with six of seven rearrangements showing similar or identical Southern blot patterns, suggesting a recombination hotspot. Southern blot analysis of 46 unrelated MPS-II patients using a full-length IDS cDNA probe; deletion breakpoint mapping with flanking markers Human molecular genetics Medium 1303177
1995 An alternative 1.4-kb IDS transcript was identified that encodes a protein identical to full-length IDS except for the absence of the 207-amino-acid C-terminal domain, which is replaced by 7 amino acids, suggesting possible existence of an additional IDS enzyme form. cDNA cloning and sequencing of alternative transcript Genomics Medium 8530090
1997 IDS is processed from two precursor forms (76 and 90 kDa) through a series of intermediate forms to mature 55- and 45-kDa polypeptides in fibroblasts, COS cells, and lymphoblastoid cell lines. Transfer of IDS from overexpressing cells to IDS-deficient cells occurs preferentially by cell-to-cell contact rather than through secreted enzyme, as IDS precursors are poorly secreted. The 76- and 62-kDa transferred IDS polypeptides are correctly processed to the mature 55- and 45-kDa forms in recipient fibroblasts. Western blotting of IDS processing in multiple cell lines; coculture and conditioned medium transfer experiments with transfected cells and IDS-deficient fibroblasts Experimental cell research Medium 9024795
1996 An intragenic IDS deletion (removing exons IV–VII) results from gene-pseudogene exchange between highly homologous regions in intron 3 and intron 7 of IDS and the IDS-2 pseudogene, producing a rearranged gene with junction intron containing pseudogene intron 3- and intron 7-related sequences. Southern blot and PCR characterization of deletion junctions; sequencing of breakpoint regions Human mutation Medium 8807335
1997 Two distinct deletions in the IDS gene region, separated by 30 kb, result from non-homologous (illegitimate) recombination events between short direct repeats. The heptamer sequence 5'-TACTCTA-3' is present at both deletion junctions, suggesting it is a recombination hotspot. Southern blot, PCR, and nucleotide sequencing of deletion junctions Genomics Medium 9244428
2006 Point mutations in the IDS gene can activate multiple cryptic splice sites. Mutations at invariant splice-site motifs (c.418+1G>C) produce only aberrantly spliced transcripts, while mutations in variant motifs (c.419G>T) or coding regions (c.245C>T) lead to a mixture of aberrant and correctly spliced transcripts. Nonsense-mediated mRNA decay (NMD) reduces mRNA levels for some aberrant transcripts. RT-PCR cloning and sequencing of transcripts; real-time RT-PCR quantification of mRNA levels; computational analysis of splice site scores Journal of molecular medicine Medium 16699754
2006 Splice mutations in the IDS gene account for ~56% of MPS II cases in a Portuguese cohort. Mutations affecting the splicing of exon 3 are particularly common, and some mutations cause dramatic changes in splicing mechanism including revelation of a cryptic exonic sequence inside intron 3. cDNA analysis is required alongside gDNA analysis to correctly classify IDS mutations. Genomic DNA sequencing and cDNA analysis by RT-PCR in 16 unrelated MPS II patients Journal of inherited metabolic disease Medium 17063374
2006 Nonsense and nonstop mutations in IDS lead to reduced mRNA levels via nonsense-mediated decay (NMD) and nonstop mRNA decay pathways, respectively, as measured by real-time RT-PCR. Two mutations (Q66X and V136fs75X) produced transcripts evading mRNA surveillance despite fulfilling the known criteria, showing variability in NMD susceptibility at the IDS locus. Real-time RT-PCR quantification of IDS mRNA levels; characterization of 17 patient alleles Biochimica et biophysica acta Medium 16495038
2009 Systemic AAV-mediated delivery of hIDS in MPSII mice results in high circulating IDS levels that cross the blood-brain barrier and correct CNS glycosaminoglycan accumulation and CNS defects. The CNS correction arises from the enzyme crossing the BBB, not from brain transduction. Systemic injection of AAV2/5CMV-hIDS in MPSII mouse pups; biochemical and histological assessment of visceral organs and brain at up to 18 months post-treatment American journal of human genetics High 19679226
2011 The synonymous IDS mutation c.879G>A (p.Gln293Gln) activates a cryptic splice site, causing a 28-bp deletion and premature termination at p.Tyr285GlufsX47, demonstrating that silent variants can cause pathogenic splicing alterations in the IDS gene. RT-PCR and Sanger sequencing of cDNA from patient fibroblasts; X-inactivation assay PloS one Medium 21829674
2011 Complex rearrangements at the IDS locus initiated by non-allelic homologous recombination between IDS and the nearby pseudogene IDSP1 (a low-copy repeat) can be resolved either by Alu-mediated recombination or by non-homologous end joining, producing partial deletion plus inverted insertion configurations. Southern blot, PCR, and in silico analysis of repetitive elements at rearrangement junctions in two MPS II patients Journal of human genetics Medium 21593745
2015 SRSF2 and hnRNP E1 may be involved respectively in activation and repression of the constitutive 3' splice site of IDS exon 3 (established by minigene and overexpression assays for mutations c.241C>T and c.257C>T). Antisense morpholino oligonucleotides and locked nucleic acids targeting the c.1122C>T aberrant splice site in exon 8 failed to abolish the abnormal transcript and instead generated additional aberrant splicing products, indicating that the oligonucleotides masked a cis-acting element required for normal 5' splice site regulation. Cell-based splicing assays with mutant minigenes; overexpression assays; transfection of antisense oligonucleotides in patient and control fibroblasts Biochimica et biophysica acta Medium 26407519
2016 Homology modeling of IDS missense variants predicts that the P120R substitution (severe MPS II) deforms an α-helix (I119–F123), causing major structural alteration, whereas N534I (attenuated MPS II) has marginal structural effect. Expression studies of nine IDS variants showed impaired enzymatic activity and aberrant intracellular processing (by immunoblotting) irrespective of phenotype. Homology modeling of IDS structure; expression studies with immunoblotting for intracellular processing; IDS enzyme activity assays in patient-derived cells Molecular genetics and metabolism Medium 27246110
2017 Expression studies of IDS variants demonstrated impaired enzymatic activity and aberrant intracellular processing; total deletion of IDS invariably results in neuronopathic MPS II phenotype. Low or cell-type-specific residual IDS activity may be sufficient to prevent the neuronal phenotype. IDS enzyme activity assays; immunoblotting for IDS protein processing; quantification of urinary glycosaminoglycans by mass spectrometry in a cohort of Dutch MPS II patients Developmental medicine and child neurology Medium 28543354
2019 In vitro expression studies of novel IDS missense mutations showed significantly decreased IDS enzymatic activity. Western blotting demonstrated that examined mutations produce IDS precursor protein at similar or slightly lower molecular mass without detectable mature forms, indicating impaired post-translational processing. Transient expression studies; IDS enzyme activity assay; Western blotting of transfected cells Clinica chimica acta Medium 30639582
2019 IDS-loaded brain-targeted PLGA nanoparticles (g7-NPs) deliver functional IDS enzyme to fibroblasts from MPS II patients, restoring IDS activity to levels comparable to healthy cells and reducing GAG content to non-pathological levels. In MPSII mice, weekly g7-NPs-IDS administration reduced GAG deposits and neuroinflammatory markers in both liver and brain tissues. In vitro IDS activity assay in patient fibroblasts; in vivo biochemical, histological, and immunohistochemical assessment in MPSII mouse model International journal of molecular sciences Medium 31022913
2010 Wild-type IDS mRNA transcripts were detected in the cDNA of three Hunter syndrome patients carrying IDS nonsense or frameshift mutations in genomic DNA, with no wild-type IDS genomic sequence detectable. Multiple methods (restriction enzyme digestion, clone sequencing, pyrosequencing, SNuPE, real-time PCR, Western blotting) confirmed both the mutant genomic sequence and the presence of some wild-type IDS protein, suggesting possible in vivo RNA-level correction of the mutations. Restriction enzyme digestion, clone sequencing, pyrosequencing, SNuPE, real-time PCR, Western blotting; segregation analysis of linked IDS polymorphism to exclude maternal X-chromosome contamination Human mutation Medium 20104590
2019 In vitro expression studies of novel IDS missense mutations (c.137A>C, c.311A>T, c.454A>C, c.797C>G, c.817C>T, c.998C>T, c.1106C>G, c.1400C>T, c.1402C>T, c.1403G>A) showed significantly decreased IDS enzymatic activity, establishing their pathogenic nature. In vitro IDS activity assay in transiently transfected cells expressing novel IDS variants International journal of molecular sciences Medium 31877959

Source papers

Stage 0 corpus · 100 papers · ranked by NIH iCite citations
Year Title Journal Citations PMID
2020 A Phase 2/3 Trial of Pabinafusp Alfa, IDS Fused with Anti-Human Transferrin Receptor Antibody, Targeting Neurodegeneration in MPS-II. Molecular therapy : the journal of the American Society of Gene Therapy 140 33038326
1995 Inversion of the IDS gene resulting from recombination with IDS-related sequences is a common cause of the Hunter syndrome. Human molecular genetics 131 7633410
2007 A mechanism for sudden infant death syndrome (SIDS): stress-induced leak via ryanodine receptors. Heart rhythm 122 17556193
2000 The nuclear receptor corepressor (N-CoR) contains three isoleucine motifs (I/LXXII) that serve as receptor interaction domains (IDs). Molecular endocrinology (Baltimore, Md.) 122 11117528
2007 Spinal cord injury-induced immune depression syndrome (SCI-IDS). The European journal of neuroscience 109 17432962
2007 IDconverter and IDClight: conversion and annotation of gene and protein IDs. BMC bioinformatics 101 17214880
2006 Inhibitors of differentiation and DNA binding (Ids) regulate Math1 and hair cell formation during the development of the organ of Corti. The Journal of neuroscience : the official journal of the Society for Neuroscience 101 16407553
1993 Sequence of the human iduronate 2-sulfatase (IDS) gene. Genomics 94 8244397
2011 A novel rare variant in SCN1Bb linked to Brugada syndrome and SIDS by combined modulation of Na(v)1.5 and K(v)4.3 channel currents. Heart rhythm 86 22155597
1977 Regulatory substances produced by lymphocytes. V. Production of inhibitor of DNA synthesis (IDS) by proliferating T lymphocytes. Journal of immunology (Baltimore, Md. : 1950) 84 300407
2017 DNA origami-based shape IDs for single-molecule nanomechanical genotyping. Nature communications 60 28382928
2016 Molecular diagnosis of 65 families with mucopolysaccharidosis type II (Hunter syndrome) characterized by 16 novel mutations in the IDS gene: Genetic, pathological, and structural studies on iduronate-2-sulfatase. Molecular genetics and metabolism 57 27246110
2000 An association between sudden infant death syndrome (SIDS) and Helicobacter pylori infection. Archives of disease in childhood 52 11040154
1999 Microbiology in sudden infant death syndrome (SIDS) and other childhood deaths. FEMS immunology and medical microbiology 52 10443492
2019 Targeting Brain Disease in MPSII: Preclinical Evaluation of IDS-Loaded PLGA Nanoparticles. International journal of molecular sciences 51 31022913
2004 IL-10 gene polymorphisms in infectious disease and SIDS. FEMS immunology and medical microbiology 50 15325397
2014 Neurochemical abnormalities in the brainstem of the Sudden Infant Death Syndrome (SIDS). Paediatric respiratory reviews 43 25304427
2012 Sarcomeric gene mutations in sudden infant death syndrome (SIDS). Forensic science international 43 22361390
1995 Presence of an IDS-related locus (IDS2) in Xq28 complicates the mutational analysis of Hunter syndrome. European journal of human genetics : EJHG 43 8528670
2013 The SCIentinel study--prospective multicenter study to define the spinal cord injury-induced immune depression syndrome (SCI-IDS)--study protocol and interim feasibility data. BMC neurology 42 24206943
2011 Analysis of the IDS gene in 38 patients with Hunter syndrome: the c.879G>A (p.Gln293Gln) synonymous variation in a female create exonic splicing. PloS one 42 21829674
2007 1-Hydroxy-2-methyl-2-(E)-butenyl 4-diphosphate reductase (IDS) is encoded by multicopy genes in gymnosperms Ginkgo biloba and Pinus taeda. Planta 42 17763867
1999 Reduction in choline acetyltransferase immunoreactivity but not muscarinic-m2 receptor immunoreactivity in the brainstem of SIDS infants. Journal of neuropathology and experimental neurology 42 10197817
2009 IDS crossing of the blood-brain barrier corrects CNS defects in MPSII mice. American journal of human genetics 40 19679226
2008 Congenital central hypoventilation syndrome (CCHS) and sudden infant death syndrome (SIDS): kindred disorders of autonomic regulation. Respiratory physiology & neurobiology 40 18579454
1997 Helix-loop-helix proteins in Schwann cells: a study of regulation and subcellular localization of Ids, REB, and E12/47 during embryonic and postnatal development. Journal of neuroscience research 38 9418957
2001 Molecular analysis of 40 Italian patients with mucopolysaccharidosis type II: New mutations in the iduronate-2-sulfatase (IDS) gene. Human mutation 36 11462244
2014 Gut microbiome in sudden infant death syndrome (SIDS) differs from that in healthy comparison babies and offers an explanation for the risk factor of prone position. International journal of medical microbiology : IJMM 35 24951305
2013 Molars and incisors: show your microarray IDs. BMC research notes 35 23531410
2013 Defining a key receptor-CheA kinase contact and elucidating its function in the membrane-bound bacterial chemosensory array: a disulfide mapping and TAM-IDS Study. Biochemistry 35 23668882
2010 Gene variants predisposing to SIDS: current knowledge. Forensic science, medicine, and pathology 34 20623341
2006 Molecular characterization of Portuguese patients with mucopolysaccharidosis type II shows evidence that the IDS gene is prone to splicing mutations. Journal of inherited metabolic disease 33 17063374
1993 The potential role of bacterial toxins in sudden infant death syndrome (SIDS). International journal of legal medicine 33 8518199
2004 Near-miss apparent SIDS from adrenal crisis. The Journal of pediatrics 32 15289763
1992 Molecular analysis of patients with Hunter syndrome: implication of a region prone to structural alterations within the IDS gene. Human molecular genetics 32 1303177
1998 Mutational spectrum of the iduronate-2-sulfatase (IDS) gene in 36 unrelated Russian MPS II patients. Human genetics 31 9921913
2017 Genotype-phenotype relationship in mucopolysaccharidosis II: predictive power of IDS variants for the neuronopathic phenotype. Developmental medicine and child neurology 30 28543354
2003 NMDA receptor 1 expression in the brainstem of human infants and its relevance to the sudden infant death syndrome (SIDS). Journal of neuropathology and experimental neurology 30 14575242
2018 An IDS-Type Sesquiterpene Synthase Produces the Pheromone Precursor (Z)-α-Bisabolene in Nezara viridula. Journal of chemical ecology 29 30267360
2006 Identification of nine new IDS alleles in mucopolysaccharidosis II. Quantitative evaluation by real-time RT-PCR of mRNAs sensitive to nonsense-mediated and nonstop decay mechanisms. Biochimica et biophysica acta 27 16495038
2007 Active caspase-3 in the sudden infant death syndrome (SIDS) brainstem. Acta neuropathologica 25 17364171
1996 IDS gene-pseudogene exchange responsible for an intragenic deletion in a Hunter patient. Human mutation 25 8807335
2011 Effects of cigarette smoke exposure on nicotinic acetylcholine receptor subunits α7 and β2 in the sudden infant death syndrome (SIDS) brainstem. Toxicology and applied pharmacology 24 22000980
2008 Sudden infant death syndrome (SIDS) in African Americans: polymorphisms in the gene encoding the stress peptide pituitary adenylate cyclase-activating polypeptide (PACAP). Acta paediatrica (Oslo, Norway : 1992) 24 19120039
1992 Susceptibility to infection in relation to SIDS. Journal of clinical pathology 23 1474153
2017 RNAseq based transcriptomics study of SMCs from carotid atherosclerotic plaque: BMP2 and IDs proteins are crucial regulators of plaque stability. Scientific reports 22 28615715
2014 Tis21 is required for adult neurogenesis in the subventricular zone and for olfactory behavior regulating cyclins, BMP4, Hes1/5 and Ids. Frontiers in cellular neuroscience 22 24744701
2008 Hypoxic-ischemic changes in SIDS brains as demonstrated by a reduction in MAP2-reactive neurons. Acta neuropathologica 22 19009302
2020 Combination of flow cytometry and molecular analysis to monitor the effect of UVC/H2O2 vs UVC/H2O2/Cu-IDS processes on pathogens and antibiotic resistant genes in secondary wastewater effluents. Water research 21 32711221
2006 Multiple cryptic splice sites can be activated by IDS point mutations generating misspliced transcripts. Journal of molecular medicine (Berlin, Germany) 20 16699754
1997 Two distinct deletions in the IDS gene and the gene W: a novel type of mutation associated with the Hunter syndrome. Genomics 20 9244428
2019 Identification and Functional Characterization of IDS Gene Mutations Underlying Taiwanese Hunter Syndrome (Mucopolysaccharidosis Type II). International journal of molecular sciences 19 31877959
2016 Positional plagiocephaly reduces parental adherence to SIDS Guidelines and inundates the health system. Child: care, health and development 19 27504717
2015 Challenges with using primer IDs to improve accuracy of next generation sequencing. PloS one 19 25741706
2009 No association of serotonin transporter gene variation with sudden infant death syndrome (SIDS) in Caucasians. Legal medicine (Tokyo, Japan) 19 19261524
2009 Myocardial inflammation, cellular death, and viral detection in sudden infant death caused by SIDS, suffocation, or myocarditis. Pediatric research 19 19287341
1993 Scottish frequency of the common G985 mutation in the medium-chain acyl-CoA dehydrogenase (MCAD) gene and the role of MCAD deficiency in sudden infant death syndrome (SIDS). Journal of inherited metabolic disease 18 8127075
2020 Explaining sudden infant death with cardiac arrhythmias: Complete exon sequencing of nine cardiac arrhythmia genes in Dutch SIDS cases highlights new and known DNA variants. Forensic science international. Genetics 17 32145446
2016 Wide allelic heterogeneity with predominance of large IDS gene complex rearrangements in a sample of Mexican patients with Hunter syndrome. Clinical genetics 17 26762690
2004 The X-linkage hypotheses for SIDS and the male excess in infant mortality. Medical hypotheses 17 15050108
1997 Enzyme activities in tissue of human benign prostatic hyperplasia after three months' treatment with the Sabal serrulata extract IDS 89 (Strogen) or placebo. European urology 17 9032543
2019 Genetic analysis of 63 Chinese patients with mucopolysaccharidosis type II: Functional characterization of seven novel IDS variants. Clinica chimica acta; international journal of clinical chemistry 16 30639582
2008 Gene therapy of Hunter syndrome: evaluation of the efficiency of muscle electro gene transfer for the production and release of recombinant iduronate-2-sulfatase (IDS). Biochimica et biophysica acta 16 18675343
2006 HLA class I epitope discovery in type 1 diabetes: independent and reproducible identification of proinsulin epitopes of CD8 T cells--report of the IDS T Cell Workshop Committee. Annals of the New York Academy of Sciences 16 17130527
1992 Metabolic deficiencies and SIDS. Journal of clinical pathology 16 1474157
2017 Pituitary adenylate cyclase activating polypeptide (PACAP) and its receptor 1 (PAC1) in the human infant brain and changes in the Sudden Infant Death Syndrome (SIDS). Neurobiology of disease 15 28392470
2015 Deep Genotyping of the IDS Gene in Colombian Patients with Hunter Syndrome. JIMD reports 15 25681085
2005 Apnea, glial apoptosis and neuronal plasticity in the arousal pathway of victims of SIDS. Forensic science international 15 15749363
2004 Ameliorative effect of IDS 30, a stinging nettle leaf extract, on chronic colitis. International journal of colorectal disease 15 15338166
1997 IDS transfer from overexpressing cells to IDS-deficient cells. Experimental cell research 15 9024795
1995 CMV-DNA detection in parenchymatous organs in cases of SIDS. International journal of legal medicine 15 7577691
2016 The Cerebellum and SIDS: Disordered Breathing in a Mouse Model of Developmental Cerebellar Purkinje Cell Loss during Recovery from Hypercarbia. Frontiers in neurology 14 27242661
2011 Significant association of TH01 allele 9.3 and SIDS. Journal of forensic sciences 14 21265843
2002 Immunosuppressant effect of IDS 30, a stinging nettle leaf extract, on myeloid dendritic cells in vitro. The Journal of rheumatology 14 11950004
2000 A polymorphism of the X-linked gene IDS increases the number of females informative for transcriptional clonality assays. American journal of hematology 14 10706761
2021 Nicotinic Receptors in the Brainstem Ascending Arousal System in SIDS With Analysis of Pre-natal Exposures to Maternal Smoking and Alcohol in High-Risk Populations of the Safe Passage Study. Frontiers in neurology 13 33776893
2015 Functional analysis of splicing mutations in the IDS gene and the use of antisense oligonucleotides to exploit an alternative therapy for MPS II. Biochimica et biophysica acta 13 26407519
1995 Identification of an alternative transcript from the human iduronate-2-sulfatase (IDS) gene. Genomics 13 8530090
2020 Post-mortem genetic investigation of cardiac disease-associated genes in sudden infant death syndrome (SIDS) cases. International journal of legal medicine 12 32789579
2009 SNP association and sequence analysis of the NOS1AP gene in SIDS. Legal medicine (Tokyo, Japan) 12 19289301
1996 Astrocytes in the hypoglossal nuclei of sudden infant death syndrome (SIDS) infants: a quantitative study. Neuropathology and applied neurobiology 12 8732189
1994 Mutations of the iduronate-2-sulfatase (IDS) gene in patients with Hunter syndrome (mucopolysaccharidosis II). Human mutation 12 7981716
2023 Altered 5-HT2A/C receptor binding in the medulla oblongata in the sudden infant death syndrome (SIDS): Part I. Tissue-based evidence for serotonin receptor signaling abnormalities in cardiorespiratory- and arousal-related circuits. Journal of neuropathology and experimental neurology 11 37226597
2017 Comparative studies of vertebrate iduronate 2-sulfatase (IDS) genes and proteins: evolution of A mammalian X-linked gene. 3 Biotech 11 28401457
2010 Enigmatic in vivo iduronate-2-sulfatase (IDS) mutant transcript correction to wild-type in Hunter syndrome. Human mutation 11 20104590
2008 Head covering and the risk for SIDS: findings from the New Zealand and German SIDS case-control studies. Pediatrics 11 18519451
2002 From epidemiology to physiology and pathology: apnea and arousal deficient theories in sudden infant death syndrome (SIDS)--with particular reference to hypoxic brainstem gliosis. Forensic science international 11 12350297
2001 Helicobacter pylori is not the cause of sudden infant death syndrome (SIDS). The American journal of gastroenterology 11 11774938
1992 Immunopathology of SIDS. Journal of clinical pathology 11 1474158
2021 Design, synthesis, and evaluation of novel coumarin-dithiocarbamate derivatives (IDs) as anti-colorectal cancer agents. Journal of enzyme inhibition and medicinal chemistry 10 33557648
2020 The α7 and β2 nicotinic acetylcholine receptor subunits regulate apoptosis in the infant hippocampus, and in sudden infant death syndrome (SIDS). Apoptosis : an international journal on programmed cell death 10 32577853
2019 Cell death in the human infant central nervous system and in sudden infant death syndrome (SIDS). Apoptosis : an international journal on programmed cell death 10 30600425
2017 Microbiome-Gut-Brain Axis: A Pathway for Improving Brainstem Serotonin Homeostasis and Successful Autoresuscitation in SIDS-A Novel Hypothesis. Frontiers in pediatrics 10 28111624
2016 Generation of Mucopolysaccharidosis type II (MPS II) human induced pluripotent stem cell (iPSC) line from a 1-year-old male with pathogenic IDS mutation. Stem cell research 10 27789399
2016 Promotion of the Unfolding Protein Response in Orexin/Dynorphin Neurons in Sudden Infant Death Syndrome (SIDS): Elevated pPERK and ATF4 Expression. Molecular neurobiology 10 27796753
2011 LCR-initiated rearrangements at the IDS locus, completed with Alu-mediated recombination or non-homologous end joining. Journal of human genetics 10 21593745
2009 Optimisation of postmortem tissue preservation and alternative protocol for serotonin transporter gene polymorphisms amplification in SIDS and SIUD cases. Experimental and molecular pathology 10 19837061
2001 Expression of apoptosis and proliferating cell nuclear antigen (PCNA) in the cardiac conduction system of crib death (SIDS). Advances in clinical pathology : the official journal of Adriatic Society of Pathology 10 11753879
2024 Investigating cardiac genetic background in sudden infant death syndrome (SIDS). International journal of legal medicine 9 38849547
2011 A novel mutation of IDS gene in a Chinese patient with mucopolysaccharidosis II by next-generation sequencing. Clinica chimica acta; international journal of clinical chemistry 9 21910981

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