Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression
about
Niemann-Pick disease type C2-hydroxypropyl-beta-cyclodextrin raises hearing threshold in normal cats and in cats with Niemann-Pick type C diseaseAutophagy in lysosomal storage disordersThe cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunctionClarifying lysosomal storage diseasesDiagnostic workup and management of patients with suspected Niemann-Pick type C diseaseNew Potential Pharmacological Functions of Chinese Herbal Medicines via Regulation of AutophagyMolecular pathways for intracellular cholesterol accumulation: common pathogenic mechanisms in Niemann-Pick disease Type C and cystic fibrosisImpairment of homeostasis in lysosomal storage disordersCollaborative development of 2-hydroxypropyl-β-cyclodextrin for the treatment of Niemann-Pick type C1 diseaseOlfactory deficits in Niemann-Pick type C1 (NPC1) diseaseGenomic expression analyses reveal lysosomal, innate immunity proteins, as disease correlates in murine models of a lysosomal storage disorderDevelopmental delay in motor skill acquisition in Niemann-Pick C1 mice reveals abnormal cerebellar morphogenesis.Hearing loss and hair cell death in mice given the cholesterol-chelating agent hydroxypropyl-β-cyclodextrinCyclodextrins as Emerging Therapeutic Tools in the Treatment of Cholesterol-Associated Vascular and Neurodegenerative DiseasesThe Potential of Cyclodextrins as Novel Active Pharmaceutical Ingredients: A Short OverviewChronic administration of an HDAC inhibitor treats both neurological and systemic Niemann-Pick type C disease in a mouse modelPre-symptomatic activation of antioxidant responses and alterations in glucose and pyruvate metabolism in Niemann-Pick Type C1-deficient murine brainCyclodextrin induces calcium-dependent lysosomal exocytosisProtein replacement therapy partially corrects the cholesterol-storage phenotype in a mouse model of Niemann-Pick type C2 diseaseActivation mobilizes the cholesterol in the late endosomes-lysosomes of Niemann Pick type C cellsGenetic and chemical activation of TFEB mediates clearance of aggregated α-synucleinPathogenic mycobacteria achieve cellular persistence by inhibiting the Niemann-Pick Type C disease cellular pathwayFostering collaborative research for rare genetic disease: the example of niemann-pick type C diseaseAllopregnanolone promotes regeneration and reduces β-amyloid burden in a preclinical model of Alzheimer's diseaseEfficacy and ototoxicity of different cyclodextrins in Niemann-Pick C diseaseDirect administration of 2-Hydroxypropyl-Beta-Cyclodextrin into guinea pig cochleae: Effects on physiological and histological measurements.Visual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ß-cyclodextrinAtypical multisensory integration in Niemann-Pick type C disease - towards potential biomarkersA novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations.Intracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease.Characterization of hydroxypropyl-beta-cyclodextrins used in the treatment of Niemann-Pick Disease type C1.Pluronic based β-cyclodextrin polyrotaxanes for treatment of Niemann-Pick Type C disease.Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration.Endocytosis of beta-cyclodextrins is responsible for cholesterol reduction in Niemann-Pick type C mutant cells.Development and validation of sensitive LC-MS/MS assays for quantification of HP-β-CD in human plasma and CSF.The development and use of small molecule inhibitors of glycosphingolipid metabolism for lysosomal storage diseases.Multi-system disorders of glycosphingolipid and ganglioside metabolismAnalytical Characterization of Methyl-β-Cyclodextrin for Pharmacological Activity to Reduce Lysosomal Cholesterol Accumulation in Niemann-Pick Disease Type C1 Cells.Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin
P2860
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P2860
Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression
description
2009 nî lūn-bûn
@nan
2009 թուականին հրատարակուած գիտական յօդուած
@hyw
2009 թվականին հրատարակված գիտական հոդված
@hy
2009年の論文
@ja
2009年論文
@yue
2009年論文
@zh-hant
2009年論文
@zh-hk
2009年論文
@zh-mo
2009年論文
@zh-tw
2009年论文
@wuu
name
Chronic cyclodextrin treatment ...... torage and disease progression
@ast
Chronic cyclodextrin treatment ...... torage and disease progression
@en
Chronic cyclodextrin treatment ...... torage and disease progression
@en-gb
Chronic cyclodextrin treatment ...... torage and disease progression
@nl
type
label
Chronic cyclodextrin treatment ...... torage and disease progression
@ast
Chronic cyclodextrin treatment ...... torage and disease progression
@en
Chronic cyclodextrin treatment ...... torage and disease progression
@en-gb
Chronic cyclodextrin treatment ...... torage and disease progression
@nl
altLabel
Chronic Cyclodextrin Treatment ...... torage and Disease Progression
@en
prefLabel
Chronic cyclodextrin treatment ...... torage and disease progression
@ast
Chronic cyclodextrin treatment ...... torage and disease progression
@en
Chronic cyclodextrin treatment ...... torage and disease progression
@en-gb
Chronic cyclodextrin treatment ...... torage and disease progression
@nl
P2093
P2860
P3181
P1433
P1476
Chronic cyclodextrin treatment ...... torage and disease progression
@en
P2093
Daniel S Ory
Gloria Stephney
Kostantin Dobrenis
Marie T Vanier
Matthew C Micsenyi
Nafeeza F Ali
Sophie Renault
Steven U Walkley
P2860
P3181
P356
10.1371/JOURNAL.PONE.0006951
P407
P577
2009-01-01T00:00:00Z