A novel cholesterol stain reveals early neuronal cholesterol accumulation in the Niemann-Pick type C1 mouse brain.
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Niemann-Pick disease type CNiemann-Pick C1 protects against atherosclerosis in mice via regulation of macrophage intracellular cholesterol traffickingPregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann-Pick C diseasePre-symptomatic activation of antioxidant responses and alterations in glucose and pyruvate metabolism in Niemann-Pick Type C1-deficient murine brainPerfringolysin O structure and mechanism of pore formation as a paradigm for cholesterol-dependent cytolysinsA novel mouse model of Niemann-Pick type C disease carrying a D1005G-Npc1 mutation comparable to commonly observed human mutations.Assessing cholesterol storage in live cells and C. elegans by stimulated Raman scattering imaging of phenyl-Diyne cholesterol.The factors affecting lipid profile in adult patients with MucopolysaccharidosisMulti-system disorders of glycosphingolipid and ganglioside metabolismLoss of Calreticulin Uncovers a Critical Role for Calcium in Regulating Cellular Lipid Homeostasis.A marked paucity of granule cells in the developing cerebellum of the Npc1(-/-) mouse is corrected by a single injection of hydroxypropyl-β-cyclodextrinCholesterol homeostatic responses provide biomarkers for monitoring treatment for the neurodegenerative disease Niemann-Pick C1 (NPC1)Improvement in lipid and protein trafficking in Niemann-Pick C1 cells by correction of a secondary enzyme defect.Niemann-Pick type C disease and intracellular cholesterol trafficking.Plasticity in membrane cholesterol contributes toward electrical maturation of hearing.Attenuation of the lysosomal death pathway by lysosomal cholesterol accumulation.GM2/GD2 and GM3 gangliosides have no effect on cellular cholesterol pools or turnover in normal or NPC1 mice.Endosomal/lysosomal processing of gangliosides affects neuronal cholesterol sequestration in Niemann-Pick disease type C.Characterisation of detergent-insoluble membranes in pollen tubes of Nicotiana tabacum (L.).Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann-Pick C1 diseaseSecondary lipid accumulation in lysosomal disease.Cholesterol metabolism and homeostasis in the brainA murine Niemann-Pick C1 I1061T knock-in model recapitulates the pathological features of the most prevalent human disease allele.Neuronal loss of Drosophila NPC1a causes cholesterol aggregation and age-progressive neurodegeneration.Identification of Niemann-Pick C1 disease biomarkers through sphingolipid profilingAllopregnanolone treatment delays cholesterol accumulation and reduces autophagic/lysosomal dysfunction and inflammation in Npc1-/- mouse brain.Partial blockage of sterol biosynthesis with a squalene synthase inhibitor in early postnatal Niemann-Pick type C npcnih null mice brains reduces neuronal cholesterol accumulation, abrogates astrogliosis, but may inhibit myelin maturation.Altered lipid metabolism in brain injury and disorders.Binding between the Niemann-Pick C1 protein and a photoactivatable cholesterol analog requires a functional sterol-sensing domain.Glymphatic distribution of CSF-derived apoE into brain is isoform specific and suppressed during sleep deprivation.Cholesterol in Niemann-Pick Type C disease.Visualization of cholesterol deposits in lysosomes of Niemann-Pick type C fibroblasts using recombinant perfringolysin O.Defective cholesterol trafficking in Niemann-Pick C-deficient cells.Complex lipid trafficking in Niemann-Pick disease type C.Trypanosoma cruzi infection results in an increase in intracellular cholesterol.Cellular Cholesterol Homeostasis in Alzheimer's Disease.Generation of a human neuronal stable cell model for niemann-pick C disease by RNA interference.Cholesterol substrate pools and steroid hormone levels are normal in the face of mutational inactivation of NPC1 protein.
P2860
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P2860
A novel cholesterol stain reveals early neuronal cholesterol accumulation in the Niemann-Pick type C1 mouse brain.
description
2004 nî lūn-bûn
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2004年の論文
@ja
2004年学术文章
@wuu
2004年学术文章
@zh-cn
2004年学术文章
@zh-hans
2004年学术文章
@zh-my
2004年学术文章
@zh-sg
2004年學術文章
@yue
2004年學術文章
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2004年學術文章
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name
A novel cholesterol stain reve ...... mann-Pick type C1 mouse brain.
@en
A novel cholesterol stain reve ...... mann-Pick type C1 mouse brain.
@nl
type
label
A novel cholesterol stain reve ...... mann-Pick type C1 mouse brain.
@en
A novel cholesterol stain reve ...... mann-Pick type C1 mouse brain.
@nl
prefLabel
A novel cholesterol stain reve ...... mann-Pick type C1 mouse brain.
@en
A novel cholesterol stain reve ...... mann-Pick type C1 mouse brain.
@nl
P2093
P2860
P1476
A novel cholesterol stain reve ...... mann-Pick type C1 mouse brain.
@en
P2093
Naomi Sakashita
Patrick C Reid
Shigeki Sugii
William F Hickey
Yoshiko Ohno-Iwashita
Yukiko Shimada
P2860
P304
P356
10.1194/JLR.D300032-JLR200
P577
2004-01-01T00:00:00Z