Perturbed myelination process of premyelinating oligodendrocyte in Niemann-Pick type C mouse.
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Tau deletion exacerbates the phenotype of Niemann-Pick type C mice and implicates autophagy in pathogenesis.The pathogenesis of Niemann-Pick type C disease: a role for autophagy?Developmental delay in motor skill acquisition in Niemann-Pick C1 mice reveals abnormal cerebellar morphogenesis.Decreased expression of myelin gene regulatory factor in Niemann-Pick type C 1 mouse"Skittish" Abca2 knockout mice display tremor, hyperactivity, and abnormal myelin ultrastructure in the central nervous system.Abnormal gene expression in cerebellum of Npc1-/- mice during postnatal development.Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrinOligodendrocyte loss during the disease course in a canine model of the lysosomal storage disease fucosidosisSize and shape of the corpus callosum in adult Niemann-Pick type C reflects state and trait illness variables.Niemann-Pick type C disease and intracellular cholesterol trafficking.Heat shock protein-based therapy as a potential candidate for treating the sphingolipidoses.Npc1 acting in neurons and glia is essential for the formation and maintenance of CNS myelin.Anatomically defined neuron-based rescue of neurodegenerative Niemann-Pick type C disorder.Defects in the retina of Niemann-pick type C 1 mutant mice.Increased apoptosis and hypomyelination in cerebral white matter of macular mutant mouse brain.Electrodiagnostic testing and histopathologic changes confirm peripheral nervous system myelin abnormalities in the feline model of niemann-pick disease type C.In vivo assessment of neurodegeneration in Niemann-Pick type C mice by quantitative T2 mapping and diffusion tensor imaging.Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice.Astrocyte-only Npc1 reduces neuronal cholesterol and triples life span of Npc1-/- mice.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.Inhibition of GM3 synthase attenuates neuropathology of Niemann-Pick disease Type C. by affecting sphingolipid metabolism.Current controversies in Niemann-Pick C1 disease: steroids or gangliosides; neurons or neurons and glia.Amelioration of enteric neuropathology in a mouse model of Niemann-Pick C by Npc1 expression in enteric gliaSevere demyelination in a patient with a late infantile form of Niemann-Pick disease type C.Down-regulation of polysialic acid is required for efficient myelin formation.Simvastatin regulates oligodendroglial process dynamics and survival.Mitogen-activated protein kinase activity may not be necessary for the neuropathology of Niemann-Pick type C mice.Lipocalin-type prostaglandin D synthase is up-regulated in oligodendrocytes in lysosomal storage diseases and binds gangliosides.
P2860
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P2860
Perturbed myelination process of premyelinating oligodendrocyte in Niemann-Pick type C mouse.
description
2004 nî lūn-bûn
@nan
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
@zh
2004年學術文章
@zh-hant
name
Perturbed myelination process ...... in Niemann-Pick type C mouse.
@en
Perturbed myelination process ...... in Niemann-Pick type C mouse.
@nl
type
label
Perturbed myelination process ...... in Niemann-Pick type C mouse.
@en
Perturbed myelination process ...... in Niemann-Pick type C mouse.
@nl
prefLabel
Perturbed myelination process ...... in Niemann-Pick type C mouse.
@en
Perturbed myelination process ...... in Niemann-Pick type C mouse.
@nl
P2093
P2860
P356
P1476
Perturbed myelination process ...... in Niemann-Pick type C mouse.
@en
P2093
Ikuko Mohri
Kinuko Suzuki
Shoichi Takikita
Takahiro Fukuda
Takashi Yagi
P2860
P304
P356
10.1093/JNEN/63.6.660
P577
2004-06-01T00:00:00Z