Progression of multiple behavioral deficits with various ages of onset in a murine model of Hurler syndrome.
about
Effects of enzyme replacement therapy started late in a murine model of mucopolysaccharidosis type IEvidence of a progressive motor dysfunction in Mucopolysaccharidosis type I mice.A novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations.Abnormal response to stress and impaired NPS-induced hyperlocomotion, anxiolytic effect and corticosterone increase in mice lacking NPSR1.Efficacy of a combined intracerebral and systemic gene delivery approach for the treatment of a severe lysosomal storage disorder.Retroviral-vector-mediated gene therapy to mucopolysaccharidosis I mice improves sensorimotor impairments and other behavioral deficits.Sensory-motor behavioral characterization of an animal model of Maroteaux-Lamy syndrome (or Mucopolysaccharidosis VI).Lysosomal storage disease: gene therapy on both sides of the blood-brain barrier.Normalization and improvement of CNS deficits in mice with Hurler syndrome after long-term peripheral delivery of BBB-targeted iduronidase.Progression of Behavioral and CNS Deficits in a Viable Murine Model of Chronic Neuronopathic Gaucher Disease.Engineering a lysosomal enzyme with a derivative of receptor-binding domain of apoE enables delivery across the blood-brain barrierEps8 controls dendritic spine density and synaptic plasticity through its actin-capping activityReprogramming erythroid cells for lysosomal enzyme production leads to visceral and CNS cross-correction in mice with Hurler syndrome.Characterization of an MPS I-H knock-in mouse that carries a nonsense mutation analogous to the human IDUA-W402X mutation.Long-term nonsense suppression therapy moderates MPS I-H disease progression.Proteomic analysis of mucopolysaccharidosis I mouse brain with two-dimensional polyacrylamide gel electrophoresis.Accumulation and distribution of α-synuclein and ubiquitin in the CNS of Gaucher disease mouse modelsSubstrate Deprivation Therapy to Reduce Glycosaminoglycan Synthesis Improves Aspects of Neurological and Skeletal Pathology in MPS I Mice.Treatment of adult MPSI mouse brains with IDUA-expressing mesenchymal stem cells decreases GAG deposition and improves exploratory behavior.Long-lasting effects of minocycline on behavior in young but not adult Fragile X mice.Pharmacological Modulation of AMPAR Rescues Intellectual Disability-Like Phenotype in Tm4sf2-/y Mice.Aversive and non-aversive memory impairment in the mucopolysaccharidosis II mouse model.An essential role for UBE2A/HR6A in learning and memory and mGLUR-dependent long-term depression.Subregional brain distribution of simple and complex glycosphingolipids in the mucopolysaccharidosis type I (Hurler syndrome) mouse: impact of diet.Comprehensive evaluation of blood-brain barrier-forming micro-vasculatures: Reference and marker genes with cellular composition.
P2860
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P2860
Progression of multiple behavioral deficits with various ages of onset in a murine model of Hurler syndrome.
description
2007 nî lūn-bûn
@nan
2007 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2007 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2007年の論文
@ja
2007年論文
@yue
2007年論文
@zh-hant
2007年論文
@zh-hk
2007年論文
@zh-mo
2007年論文
@zh-tw
2007年论文
@wuu
name
Progression of multiple behavi ...... rine model of Hurler syndrome.
@ast
Progression of multiple behavi ...... rine model of Hurler syndrome.
@en
type
label
Progression of multiple behavi ...... rine model of Hurler syndrome.
@ast
Progression of multiple behavi ...... rine model of Hurler syndrome.
@en
prefLabel
Progression of multiple behavi ...... rine model of Hurler syndrome.
@ast
Progression of multiple behavi ...... rine model of Hurler syndrome.
@en
P2093
P2860
P1433
P1476
Progression of multiple behavi ...... urine model of Hurler syndrome
@en
P2093
Anthony Sciascia
Charles V Vorhees
P2860
P304
P356
10.1016/J.BRAINRES.2007.10.036
P407
P577
2007-10-23T00:00:00Z