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Search results 1 to 100 out of 222 for Idua

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Type Details Score
Gene
Type: gene
Organism: human
Gene
Type: gene
Organism: zebrafish
Gene
Type: gene
Organism: frog, western clawed
Gene
Type: gene
Organism: macaque, rhesus
Gene
Type: gene
Organism: cat, domestic
Gene
Type: gene
Organism: rat
Gene
Type: gene
Organism: cattle
Gene
Type: gene
Organism: dog, domestic
Gene
Type: gene
Organism: chicken
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Publication
First Author: Keeling KM
Year: 2013
Journal: PLoS One
Title: Attenuation of nonsense-mediated mRNA decay enhances in vivo nonsense suppression.
Volume: 8
Issue: 4
Pages: e60478
Publication
First Author: Bose SK
Year: 2021
Journal: Nat Commun
Title: In utero adenine base editing corrects multi-organ pathology in a lethal lysosomal storage disease.
Volume: 12
Issue: 1
Pages: 4291
Publication
First Author: Scott HS
Year: 1990
Journal: Am J Hum Genet
Title: Chromosomal localization of the human alpha-L-iduronidase gene (IDUA) to 4p16.3.
Volume: 47
Issue: 5
Pages: 802-7
Publication
First Author: Liu Y
Year: 2005
Journal: Mol Ther
Title: Liver-directed neonatal gene therapy prevents cardiac, bone, ear, and eye disease in mucopolysaccharidosis I mice.
Volume: 11
Issue: 1
Pages: 35-47
Publication
First Author: Clarke LA
Year: 1997
Journal: Hum Mol Genet
Title: Murine mucopolysaccharidosis type I: targeted disruption of the murine alpha-L-iduronidase gene.
Volume: 6
Issue: 4
Pages: 503-11
Publication
First Author: Kuehn SC
Year: 2015
Journal: Hum Mol Genet
Title: Impaired bone remodeling and its correction by combination therapy in a mouse model of mucopolysaccharidosis-I.
Volume: 24
Issue: 24
Pages: 7075-86
Publication
First Author: Dai M
Year: 2014
Journal: Proc Natl Acad Sci U S A
Title: Platelets are efficient and protective depots for storage, distribution, and delivery of lysosomal enzyme in mice with Hurler syndrome.
Volume: 111
Issue: 7
Pages: 2680-5
Publication
First Author: Wang D
Year: 2009
Journal: Proc Natl Acad Sci U S A
Title: Reprogramming erythroid cells for lysosomal enzyme production leads to visceral and CNS cross-correction in mice with Hurler syndrome.
Volume: 106
Issue: 47
Pages: 19958-63
Publication  
First Author: Mendez DC
Year: 2015
Journal: Mol Ther Methods Clin Dev
Title: A novel, long-lived, and highly engraftable immunodeficient mouse model of mucopolysaccharidosis type I.
Volume: 2
Pages: 14068
Publication
First Author: Kreutz F
Year: 2013
Journal: Gene
Title: Alterations of membrane lipids and in gene expression of ganglioside metabolism in different brain structures in a mouse model of mucopolysaccharidosis type I (MPS I).
Volume: 527
Issue: 1
Pages: 109-14
Publication
First Author: Chung S
Year: 2007
Journal: Mol Genet Metab
Title: Effect of neonatal administration of a retroviral vector expressing alpha-L-iduronidase upon lysosomal storage in brain and other organs in mucopolysaccharidosis I mice.
Volume: 90
Issue: 2
Pages: 181-92
Publication
First Author: Barbosa Mendes A
Year: 2019
Journal: PLoS One
Title: Sexual behaviour in a murine model of mucopolysaccharidosis type I (MPS I).
Volume: 14
Issue: 12
Pages: e0220429
Publication
First Author: Baldo G
Year: 2013
Journal: Mol Genet Metab
Title: Enzyme replacement therapy started at birth improves outcome in difficult-to-treat organs in mucopolysaccharidosis I mice.
Volume: 109
Issue: 1
Pages: 33-40
Publication
First Author: Martin PK
Year: 2014
Journal: PLoS One
Title: Mesenchymal stem cells do not prevent antibody responses against human α-L-iduronidase when used to treat mucopolysaccharidosis type I.
Volume: 9
Issue: 3
Pages: e92420
Publication
First Author: Wang D
Year: 2010
Journal: Mol Genet Metab
Title: Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.
Volume: 99
Issue: 1
Pages: 62-71
Publication
First Author: Koizumi T
Year: 1992
Journal: Mamm Genome
Title: Linkage, but not gene order, of homologous loci, including alpha-L-iduronidase (Idua), is conserved in the Huntington disease region of the mouse and human genomes.
Volume: 3
Issue: 1
Pages: 23-7
Protein
Organism: Mus musculus/domesticus
Length: 643  
Fragment?: false
Publication
First Author: Garcia-Rivera MF
Year: 2007
Journal: Brain Res Bull
Title: Characterization of an immunodeficient mouse model of mucopolysaccharidosis type I suitable for preclinical testing of human stem cell and gene therapy.
Volume: 74
Issue: 6
Pages: 429-38
Publication  
First Author: Viana GM
Year: 2020
Journal: Int J Mol Sci
Title: Cathepsin B-associated Activation of Amyloidogenic Pathway in Murine Mucopolysaccharidosis Type I Brain Cortex.
Volume: 21
Issue: 4
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus caroli
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: mouse, laboratory
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus pahari
Protein Coding Gene
Type: protein_coding_gene
Organism: Mus spretus
Publication
First Author: Clarke LA
Year: 1994
Journal: Genomics
Title: Murine alpha-L-iduronidase: cDNA isolation and expression.
Volume: 24
Issue: 2
Pages: 311-6
Publication
First Author: Pasqualim G
Year: 2015
Journal: PLoS One
Title: Effects of enzyme replacement therapy started late in a murine model of mucopolysaccharidosis type I.
Volume: 10
Issue: 2
Pages: e0117271
Publication
First Author: Jordan MC
Year: 2005
Journal: Mol Genet Metab
Title: Cardiac manifestations in the mouse model of mucopolysaccharidosis I.
Volume: 86
Issue: 1-2
Pages: 233-43
Publication
First Author: Satzer D
Year: 2015
Journal: PLoS One
Title: Assessment of dysmyelination with RAFFn MRI: application to murine MPS I.
Volume: 10
Issue: 2
Pages: e0116788
Publication
First Author: Wolf DA
Year: 2012
Journal: Mol Genet Metab
Title: Lysosomal enzyme can bypass the blood-brain barrier and reach the CNS following intranasal administration.
Volume: 106
Issue: 1
Pages: 131-4
Publication
First Author: Reolon GK
Year: 2006
Journal: Brain Res
Title: Long-term memory for aversive training is impaired in Idua(-/-) mice, a genetic model of mucopolysaccharidosis type I.
Volume: 1076
Issue: 1
Pages: 225-30
Publication
First Author: Zhu W
Year: 2023
Journal: Sci Rep
Title: Mapping brain networks in MPS I mice and their restoration following gene therapy.
Volume: 13
Issue: 1
Pages: 12716
Publication
First Author: Graupman P
Year: 2004
Journal: J Craniofac Surg
Title: Craniofacial abnormalities in a murine knock-out model of mucopolysaccharidosis I H: a computed tomography and anatomic study.
Volume: 15
Issue: 3
Pages: 392-8
Publication
First Author: Schachern PA
Year: 2007
Journal: Int J Pediatr Otorhinolaryngol
Title: Age-related functional and histopathological changes of the ear in the MPS I mouse.
Volume: 71
Issue: 2
Pages: 197-203
Publication
First Author: Baldo G
Year: 2012
Journal: Behav Brain Res
Title: Evidence of a progressive motor dysfunction in Mucopolysaccharidosis type I mice.
Volume: 233
Issue: 1
Pages: 169-75
Publication
First Author: Wang J
Year: 2022
Journal: Nature
Title: AAV-delivered suppressor tRNA overcomes a nonsense mutation in mice.
Volume: 604
Issue: 7905
Pages: 343-348
Publication
First Author: Makino E
Year: 2018
Journal: Sci Rep
Title: Fast, sensitive method for trisaccharide biomarker detection in mucopolysaccharidosis type 1.
Volume: 8
Issue: 1
Pages: 3681
Publication
First Author: Gomez-Ospina N
Year: 2019
Journal: Nat Commun
Title: Human genome-edited hematopoietic stem cells phenotypically correct Mucopolysaccharidosis type I.
Volume: 10
Issue: 1
Pages: 4045
Publication  
First Author: Malinowska M
Year: 2024
Journal: Int J Mol Sci
Title: Efficacy of a Combination Therapy with Laronidase and Genistein in Treating Mucopolysaccharidosis Type I in a Mouse Model.
Volume: 25
Issue: 4
Publication  
First Author: Pan D
Year: 2008
Journal: Brain Res
Title: Progression of multiple behavioral deficits with various ages of onset in a murine model of Hurler syndrome.
Volume: 1188
Pages: 241-53
Publication
First Author: Watson HA
Year: 2014
Journal: J Biol Chem
Title: Heparan sulfate inhibits hematopoietic stem and progenitor cell migration and engraftment in mucopolysaccharidosis I.
Volume: 289
Issue: 52
Pages: 36194-203
Publication
First Author: Holley RJ
Year: 2011
Journal: J Biol Chem
Title: Mucopolysaccharidosis type I, unique structure of accumulated heparan sulfate and increased N-sulfotransferase activity in mice lacking α-l-iduronidase.
Volume: 286
Issue: 43
Pages: 37515-24
Publication
First Author: Pievani A
Year: 2015
Journal: Blood
Title: Neonatal bone marrow transplantation prevents bone pathology in a mouse model of mucopolysaccharidosis type I.
Volume: 125
Issue: 10
Pages: 1662-71
Publication
First Author: Randall DR
Year: 2008
Journal: Mol Genet Metab
Title: Heparin cofactor II-thrombin complex: a biomarker of MPS disease.
Volume: 94
Issue: 4
Pages: 456-61
Publication  
First Author: Gaffke L
Year: 2023
Journal: Biochem Biophys Res Commun
Title: Contribution of vesicle trafficking dysregulation to the pathomechanism of mucopolysaccharidosis.
Volume: 665
Pages: 107-117
Publication  
First Author: Gaffke L
Year: 2023
Journal: Cells
Title: Actin Cytoskeleton Polymerization and Focal Adhesion as Important Factors in the Pathomechanism and Potential Targets of Mucopolysaccharidosis Treatment.
Volume: 12
Issue: 13
Publication
First Author: Khalid O
Year: 2016
Journal: PLoS One
Title: Immune-Mediated Inflammation May Contribute to the Pathogenesis of Cardiovascular Disease in Mucopolysaccharidosis Type I.
Volume: 11
Issue: 3
Pages: e0150850
Publication  
First Author: Kingma SD
Year: 2015
Journal: JIMD Rep
Title: Adverse Effects of Genistein in a Mucopolysaccharidosis Type I Mouse Model.
Volume: 23
Pages: 77-83
Publication
First Author: Heppner JM
Year: 2015
Journal: Mol Genet Metab
Title: Extracellular matrix disruption is an early event in the pathogenesis of skeletal disease in mucopolysaccharidosis I.
Volume: 114
Issue: 2
Pages: 146-55
Publication  
First Author: Saville JT
Year: 2016
Journal: Exp Neurol
Title: Selective normalisation of regional brain bis(monoacylglycero)phosphate in the mucopolysaccharidosis 1 (Hurler) mouse.
Volume: 277
Pages: 68-75
Publication
First Author: Saville JT
Year: 2017
Journal: J Neurochem
Title: Subregional brain distribution of simple and complex glycosphingolipids in the mucopolysaccharidosis type I (Hurler syndrome) mouse: impact of diet.
Volume: 141
Issue: 2
Pages: 287-295
Publication
First Author: Palpant NJ
Year: 2011
Journal: Am J Physiol Heart Circ Physiol
Title: Cardiac disease in mucopolysaccharidosis type I attributed to catecholaminergic and hemodynamic deficiencies.
Volume: 300
Issue: 1
Pages: H356-65
Publication
First Author: Dai M
Year: 2018
Journal: PLoS One
Title: Comprehensive evaluation of blood-brain barrier-forming micro-vasculatures: Reference and marker genes with cellular composition.
Volume: 13
Issue: 5
Pages: e0197379
Publication
First Author: Russell C
Year: 1998
Journal: Clin Genet
Title: Murine MPS I: insights into the pathogenesis of Hurler syndrome.
Volume: 53
Issue: 5
Pages: 349-61
Publication
First Author: Azario I
Year: 2017
Journal: Sci Rep
Title: Neonatal umbilical cord blood transplantation halts skeletal disease progression in the murine model of MPS-I.
Volume: 7
Issue: 1
Pages: 9473
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:3516422
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1716122
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1691622
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1760622
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1737322
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:3284222
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1666522
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1619822
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression    
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1754922
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Note: Expression was not detected in the corpus striatum (lateral ganglionic eminence).
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1697422
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression    
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:3276322
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Note: Expression was not detected in the face/soft tissues.
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1783822
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1665922
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1706822
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1674822
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1670622
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1738222
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1654222
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1777922
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1684922
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1716822
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1
GXD Expression      
Probe: MGI:5310238
Assay Type: RNA in situ
Annotation Date: 2012-06-12
Strength: Absent
Sex: Not Specified
Emaps: EMAPS:1619322
Stage: TS22
Assay Id: MGI:5423562
Age: embryonic day 14.5
Specimen Label: EB524; Specimen B351
Detected: false
Specimen Num: 1