Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice.
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The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunctionDisruption in connexin-based communication is associated with intracellular Ca²⁺ signal alterations in astrocytes from Niemann-Pick type C miceGenomic expression analyses reveal lysosomal, innate immunity proteins, as disease correlates in murine models of a lysosomal storage disorderPre-symptomatic activation of antioxidant responses and alterations in glucose and pyruvate metabolism in Niemann-Pick Type C1-deficient murine brainVisual evoked potentials of Niemann-Pick type C1 mice reveal an impairment of the visual pathway that is rescued by 2-hydroxypropyl-ß-cyclodextrinNiemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrinBehavioral deficits, early gliosis, dysmyelination and synaptic dysfunction in a mouse model of mucolipidosis IV.Defects in the retina of Niemann-pick type C 1 mutant mice.A murine Niemann-Pick C1 I1061T knock-in model recapitulates the pathological features of the most prevalent human disease allele.Neuronal and epithelial cell rescue resolves chronic systemic inflammation in the lipid storage disorder Niemann-Pick C.Neuroinflammatory paradigms in lysosomal storage diseases.Clinically early-stage CSPα mutation carrier exhibits remarkable terminal stage neuronal pathology with minimal evidence of synaptic loss.Quantitative proteomic analysis of Niemann-Pick disease, type C1 cerebellum identifies protein biomarkers and provides pathological insight.Immune dysfunction in Niemann-Pick disease type C.Human and mouse neuroinflammation markers in Niemann-Pick disease, type C1.Plasma signature of neurological disease in the monogenetic disorder Niemann-Pick Type C.Mechanisms underlying synaptic vulnerability and degeneration in neurodegenerative disease.The neuropsychiatry of inborn errors of metabolism.Role of endolysosomes and cholesterol in the pathogenesis of Alzheimer's disease: Insights into why statins might not provide clinical benefit.Synaptic failure: The achilles tendon of sphingolipidosesSynergistic effects of treating the spinal cord and brain in CLN1 disease.Systemic AAV9 gene therapy improves the lifespan of mice with Niemann-Pick disease, type C1.Circadian profiling in two mouse models of lysosomal storage disorders; Niemann Pick type-C and Sandhoff disease.Glial cells are functionally impaired in juvenile neuronal ceroid lipofuscinosis and detrimental to neurons.Role of LDL cholesterol and endolysosomes in amyloidogenesis and Alzheimer's disease.Cyclodextrin Alters GABAergic Input to CA1 Pyramidal Cells in Wild-Type But Not in NPC1-Deficient Mice.Altered Cholesterol Intracellular Trafficking and the Development of Pathological Hallmarks of Sporadic AD.Severe demyelination in a patient with a late infantile form of Niemann-Pick disease type C.Acid Ceramidase Deficiency in Mice Results in a Broad Range of Central Nervous System Abnormalities.Recent neuroimaging, neurophysiological, and neuropathological advances for the understanding of NPC.
P2860
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P2860
Early glial activation, synaptic changes and axonal pathology in the thalamocortical system of Niemann-Pick type C1 mice.
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
Early glial activation, synapt ...... of Niemann-Pick type C1 mice.
@en
type
label
Early glial activation, synapt ...... of Niemann-Pick type C1 mice.
@en
prefLabel
Early glial activation, synapt ...... of Niemann-Pick type C1 mice.
@en
P2093
P2860
P1476
Early glial activation, synapt ...... of Niemann-Pick type C1 mice.
@en
P2093
Andrew M S Wong
David A Smith
Sarah N R Pressey
P2860
P304
P356
10.1016/J.NBD.2011.12.027
P577
2011-12-16T00:00:00Z