Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations.
about
Molecular pathways for intracellular cholesterol accumulation: common pathogenic mechanisms in Niemann-Pick disease Type C and cystic fibrosisCollaborative development of 2-hydroxypropyl-β-cyclodextrin for the treatment of Niemann-Pick type C1 diseaseThe lysosome: from waste bag to potential therapeutic targetActivation mobilizes the cholesterol in the late endosomes-lysosomes of Niemann Pick type C cellsEfficacy and ototoxicity of different cyclodextrins in Niemann-Pick C diseaseVisual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ß-cyclodextrinIntracisternal cyclodextrin prevents cerebellar dysfunction and Purkinje cell death in feline Niemann-Pick type C1 disease.Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrinTherapeutic potential of cyclodextrins in the treatment of Niemann-Pick type C disease.Hepatic entrapment of esterified cholesterol drives continual expansion of whole body sterol pool in lysosomal acid lipase-deficient miceCholesterol homeostatic responses provide biomarkers for monitoring treatment for the neurodegenerative disease Niemann-Pick C1 (NPC1)Synthesis, characterization, and evaluation of pluronic-based β-cyclodextrin polyrotaxanes for mobilization of accumulated cholesterol from Niemann-Pick type C fibroblasts.Systemic administration of 2-hydroxypropyl-β-cyclodextrin to symptomatic Npc1-deficient mice slows cholesterol sequestration in the major organs and improves liver functionTissue-specific knockouts of ACAT2 reveal that intestinal depletion is sufficient to prevent diet-induced cholesterol accumulation in the liver and blood.Weekly Treatment of 2-Hydroxypropyl-β-cyclodextrin Improves Intracellular Cholesterol Levels in LDL Receptor Knockout MiceCyclodextrin mediates rapid changes in lipid balance in Npc1-/- mice without carrying cholesterol through the bloodstream.Neuroprotection by cyclodextrin in cell and mouse models of Alzheimer diseaseOntogenic changes in lung cholesterol metabolism, lipid content, and histology in mice with Niemann-Pick type C disease.Ezetimibe markedly attenuates hepatic cholesterol accumulation and improves liver function in the lysosomal acid lipase-deficient mouse, a model for cholesteryl ester storage disease.Plasma signature of neurological disease in the monogenetic disorder Niemann-Pick Type C.Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment.Sterol transfer between cyclodextrin and membranes: similar but not identical mechanism to NPC2-mediated cholesterol transfer.Modeling the effects of cyclodextrin on intracellular membrane vesicles from Cos-7 cells prepared by sonication and carbonate treatment.2-hydroxypropyl-β-cyclodextrins and the Blood-Brain Barrier: Considerations for Niemann-Pick Disease Type C1.Impact of loss of SOAT2 function on disease progression in the lysosomal acid lipase-deficient mouse.Lipid biomarkers for the peroxisomal and lysosomal disorders: their formation, metabolism and measurement.The extending spectrum of NPC1-related human disorders: from Niemann-Pick C1 Disease to obesity.Tolerance of chronic HDACi treatment for neurological, visceral and lung Niemann-Pick Type C disease in mice.
P2860
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P2860
Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations.
description
2011 nî lūn-bûn
@nan
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
2011年论文
@zh
2011年论文
@zh-cn
name
Quantitative role of LAL, NPC2 ...... pharmacological manipulations.
@ast
Quantitative role of LAL, NPC2 ...... pharmacological manipulations.
@en
type
label
Quantitative role of LAL, NPC2 ...... pharmacological manipulations.
@ast
Quantitative role of LAL, NPC2 ...... pharmacological manipulations.
@en
prefLabel
Quantitative role of LAL, NPC2 ...... pharmacological manipulations.
@ast
Quantitative role of LAL, NPC2 ...... pharmacological manipulations.
@en
P2093
P2860
P356
P1476
Quantitative role of LAL, NPC2 ...... pharmacological manipulations.
@en
P2093
Anna M Taylor
Charina M Ramirez
John M Dietschy
Joyce J Repa
Stephen D Turley
P2860
P304
P356
10.1194/JLR.M013789
P577
2011-02-02T00:00:00Z