Glycosaminoglycans

iduronidase, alpha-L ; Mus musculus







39 Article(s)
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1 33145772 Cardiac pathology in mucopolysaccharidosis I mice: Losartan modifies ERK1/2 activation during cardiac remodeling. 2021 May 1
2 33319323 Morphological damage in Sertoli, myoid and interstitial cells in a mouse model of mucopolysaccharidosis type I (MPS I). 2021 Jan 4
3 33434858 Biomechanical and histological characterization of MPS I mice femurs. 2021 Feb 2
4 31679559 Evidence that glycosaminoglycan storage and collagen deposition in the cauda epididymidis does not impair sperm viability in the Mucopolysaccharidosis type I mouse model. 2020 Feb 3
5 32162173 Morphologic description of male reproductive accessory glands in a mouse model of mucopolysaccharidosis type I (MPS I). 2020 Apr 1
6 32439268 Neonatal combination therapy improves some of the clinical manifestations in the Mucopolysaccharidosis type I murine model. 2020 Jul 1
7 30528089 ZFN-Mediated In Vivo Genome Editing Corrects Murine Hurler Syndrome. 2019 Jan 2 3
8 31065277 Induced Pluripotent Stem Cell Derivation and Ex Vivo Gene Correction Using a Mucopolysaccharidosis Type 1 Disease Mouse Model. 2019 2
9 31834922 Sexual behaviour in a murine model of mucopolysaccharidosis type I (MPS I). 2019 2
10 28882767 Optimization of alginate microcapsules containing cells overexpressing α-l-iduronidase using Box-Behnken design. 2018 Jan 1 4
11 29198892 RTB lectin-mediated delivery of lysosomal α-l-iduronidase mitigates disease manifestations systemically including the central nervous system. 2018 Feb 1
12 29940298 Intra-articular nonviral gene therapy in mucopolysaccharidosis I mice. 2018 Sep 5 2
13 28150116 Subcutaneous implantation of microencapsulated cells overexpressing α-L-iduronidase for mucopolysaccharidosis type I treatment. 2017 Mar 4
14 28585336 Liver-Directed Human Amniotic Epithelial Cell Transplantation Improves Systemic Disease Phenotype in Hurler Syndrome Mouse Model. 2017 Jul 2
15 28958576 Guanidinylated Neomycin Conjugation Enhances Intranasal Enzyme Replacement in the Brain. 2017 Dec 6 2
16 27720939 Differential expression of microRNAs from miR-17 family in the cerebellum of mucopolysaccharidosis type I mice. 2016 Dec 31 1
17 25541102 Shotgun proteomics reveals possible mechanisms for cognitive impairment in Mucopolysaccharidosis I mice. 2015 Feb 4
18 26407983 Decreased performance in IDUA knockout mouse mimic limitations of joint function and locomotion in patients with Hurler syndrome. 2015 Sep 25 1
19 28649535 Characterization of the MPS I-H knock-in mouse reveals increased femoral biomechanical integrity with compromised material strength and altered bone geometry. 2015 Dec 1
20 22983812 Retroviral-vector-mediated gene therapy to mucopolysaccharidosis I mice improves sensorimotor impairments and other behavioral deficits. 2013 May 4
21 23593225 Attenuation of nonsense-mediated mRNA decay enhances in vivo nonsense suppression. 2013 2
22 22056610 The designer aminoglycoside NB84 significantly reduces glycosaminoglycan accumulation associated with MPS I-H in the Idua-W392X mouse. 2012 Jan 3
23 22520214 Treatment of adult MPSI mouse brains with IDUA-expressing mesenchymal stem cells decreases GAG deposition and improves exploratory behavior. 2012 Apr 20 5
24 21081900 Minicircle DNA-based gene therapy coupled with immune modulation permits long-term expression of α-L-iduronidase in mice with mucopolysaccharidosis type I. 2011 Mar 1
25 21253856 Lipid composition of whole brain and cerebellum in Hurler syndrome (MPS IH) mice. 2011 Sep 1
26 21965007 Genomic instability in blood cells from murine model of mucopolysaccharidosis type I. 2011 Dec 1
27 20847202 Gene therapy augments the efficacy of hematopoietic cell transplantation and fully corrects mucopolysaccharidosis type I phenotype in the mouse model. 2010 Dec 9 1
28 19384290 Systemic correction of storage disease in MPS I NOD/SCID mice using the sleeping beauty transposon system. 2009 Jul 2
29 18613275 Improved retroviral vector design results in sustained expression after adult gene therapy in mucopolysaccharidosis I mice. 2008 Sep 4
30 16979922 Effect of neonatal administration of a retroviral vector expressing alpha-L-iduronidase upon lysosomal storage in brain and other organs in mucopolysaccharidosis I mice. 2007 Feb 2
31 17311010 Improvements in mucopolysaccharidosis I mice after adult retroviral vector-mediated gene therapy with immunomodulation. 2007 May 2
32 17920451 Characterization of an immunodeficient mouse model of mucopolysaccharidosis type I suitable for preclinical testing of human stem cell and gene therapy. 2007 Nov 1 4
33 17044753 Limited transgene immune response and long-term expression of human alpha-L-iduronidase in young adult mice with mucopolysaccharidosis type I by liver-directed gene therapy. 2006 Nov 2
34 15585404 Liver-directed neonatal gene therapy prevents cardiac, bone, ear, and eye disease in mucopolysaccharidosis I mice. 2005 Jan 2
35 15703491 Gene therapy for a mucopolysaccharidosis type I murine model with lentiviral-IDUA vector. 2005 Jan 1
36 15851016 Neonatal gene therapy of MPS I mice by intravenous injection of a lentiviral vector. 2005 May 1
37 16435197 Nonviral in vivo gene transfer in the mucopolysaccharidosis I murine model. 2005 1
38 12948739 Treatment of the mouse model of mucopolysaccharidosis I with retrovirally transduced bone marrow. 2003 Aug 1
39 9097952 Murine mucopolysaccharidosis type I: targeted disruption of the murine alpha-L-iduronidase gene. 1997 Apr 1