Niemann-Pick type C 1 function requires lumenal domain residues that mediate cholesterol-dependent NPC2 binding
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Ebola virus entry requires the host-programmed recognition of an intracellular receptorGenetic dissection of a cell-autonomous neurodegenerative disorder: lessons learned from mouse models of Niemann-Pick disease type CMapping of Ebolavirus Neutralization by Monoclonal Antibodies in the ZMapp Cocktail Using Cryo-Electron Tomography and Studies of Cellular EntryVisual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ß-cyclodextrinA new player in the puzzle of filovirus entry.Adaptations of energy metabolism associated with increased levels of mitochondrial cholesterol in Niemann-Pick type C1-deficient cells.Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrinTherapeutic potential of cyclodextrins in the treatment of Niemann-Pick type C disease.A marked paucity of granule cells in the developing cerebellum of the Npc1(-/-) mouse is corrected by a single injection of hydroxypropyl-β-cyclodextrinScientific side trips: six excursions from the beaten path.A human genome-wide screen for regulators of clathrin-coated vesicle formation reveals an unexpected role for the V-ATPase.Insights into the mechanisms of sterol transport between organelles.Npc1 acting in neurons and glia is essential for the formation and maintenance of CNS myelin.Pulmonary abnormalities in animal models due to Niemann-Pick type C1 (NPC1) or C2 (NPC2) diseaseDesign and synthesis of a crosslinker for studying intracellular steroid trafficking pathways.Lysosomal dysfunction causes neurodegeneration in mucolipidosis II 'knock-in' mice.Cyclodextrin mediates rapid changes in lipid balance in Npc1-/- mice without carrying cholesterol through the bloodstream.Shortened primary cilium length and dysregulated Sonic hedgehog signaling in Niemann-Pick C1 disease.Quantitative comparison of the efficacy of various compounds in lowering intracellular cholesterol levels in Niemann-Pick type C fibroblasts.Glycosylation inhibition reduces cholesterol accumulation in NPC1 protein-deficient cellsQuantitative magnetic resonance imaging of brain atrophy in a mouse model of Niemann-Pick type C disease.δ-Tocopherol reduces lipid accumulation in Niemann-Pick type C1 and Wolman cholesterol storage disorders.Filovirus receptor NPC1 contributes to species-specific patterns of ebolavirus susceptibility in batsIdentification of NPC1 as the target of U18666A, an inhibitor of lysosomal cholesterol export and Ebola infectionA rare case of Niemann-Pick disease type C without neurological involvement in a 66-year-old patient.The cytosolic adaptor AP-1A is essential for the trafficking and function of Niemann-Pick type C proteinsA Single Residue in Ebola Virus Receptor NPC1 Influences Cellular Host Range in Reptiles.Clues to NPC1-mediated cholesterol export from lysosomes.Structure of human Niemann-Pick C1 protein.Clues to the mechanism of cholesterol transfer from the structure of NPC1 middle lumenal domain bound to NPC2.Adenovirus RIDα uncovers a novel pathway requiring ORP1L for lipid droplet formation independent of NPC1.The potential of histone deacetylase inhibitors in Niemann - Pick type C disease.Triazoles inhibit cholesterol export from lysosomes by binding to NPC1.Role of endosomes and lysosomes in human disease.Endosomal cholesterol trafficking: protein factors at a glance.Current controversies in Niemann-Pick C1 disease: steroids or gangliosides; neurons or neurons and glia.Treatment of Niemann--pick type C disease by histone deacetylase inhibitors.Complex lipid trafficking in Niemann-Pick disease type C.3.3 Å structure of Niemann-Pick C1 protein reveals insights into the function of the C-terminal luminal domain in cholesterol transport.Histone deacetylase inhibitors correct the cholesterol storage defect in most Niemann-Pick C1 mutant cells.
P2860
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P2860
Niemann-Pick type C 1 function requires lumenal domain residues that mediate cholesterol-dependent NPC2 binding
description
2011 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած
@hyw
2011 թվականի նոյեմբերին հրատարակված գիտական հոդված
@hy
article publié dans les Procee ...... f the United States of America
@fr
artículu científicu espublizáu en 2011
@ast
im November 2011 veröffentlichter wissenschaftlicher Artikel
@de
scientific journal article
@en
vedecký článok (publikovaný 2011/11/22)
@sk
vědecký článek publikovaný v roce 2011
@cs
wetenschappelijk artikel (gepubliceerd op 2011/11/22)
@nl
наукова стаття, опублікована в листопаді 2011
@uk
name
Niemann-Pick type C 1 function ...... esterol-dependent NPC2 binding
@ast
Niemann-Pick type C 1 function ...... esterol-dependent NPC2 binding
@en
Niemann-Pick type C 1 function ...... esterol-dependent NPC2 binding
@nl
type
label
Niemann-Pick type C 1 function ...... esterol-dependent NPC2 binding
@ast
Niemann-Pick type C 1 function ...... esterol-dependent NPC2 binding
@en
Niemann-Pick type C 1 function ...... esterol-dependent NPC2 binding
@nl
prefLabel
Niemann-Pick type C 1 function ...... esterol-dependent NPC2 binding
@ast
Niemann-Pick type C 1 function ...... esterol-dependent NPC2 binding
@en
Niemann-Pick type C 1 function ...... esterol-dependent NPC2 binding
@nl
P2860
P3181
P356
P1476
Niemann-Pick type C 1 function ...... esterol-dependent NPC2 binding
@en
P2093
Maika S. Deffieu
Suzanne R. Pfeffer
P2860
P304
18932–18936
P3181
P356
10.1073/PNAS.1110439108
P407
P577
2011-11-22T00:00:00Z