Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C disease - Wikidata - thesis-toulmin - TriplyDB

Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C disease

Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C disease

http://www.wikidata.org/entity/Q24811579

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Tau deletion exacerbates the phenotype of Niemann-Pick type C mice and implicates autophagy in pathogenesis.Biallelic mutations in SNX14 cause a syndromic form of cerebellar atrophy and lysosome-autophagosome dysfunction Autophagy in lysosomal storage disorders The pathogenesis of Niemann-Pick type C disease: a role for autophagy?Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann-Pick C disease Genetic dissection of a cell-autonomous neurodegenerative disorder: lessons learned from mouse models of Niemann-Pick disease type C Ataxia is the major neuropathological finding in arylsulfatase G-deficient mice: similarities and dissimilarities to Sanfilippo disease (mucopolysaccharidosis type III).Disruption in connexin-based communication is associated with intracellular Ca²⁺ signal alterations in astrocytes from Niemann-Pick type C mice ATP13A2 regulates mitochondrial bioenergetics through macroautophagy Pre-symptomatic activation of antioxidant responses and alterations in glucose and pyruvate metabolism in Niemann-Pick Type C1-deficient murine brain Expression and lysosomal targeting of CLN7, a major facilitator superfamily transporter associated with variant late-infantile neuronal ceroid lipofuscinosis Chronic exposure to low frequency noise at moderate levels causes impaired balance in mice.Extensive macrophage accumulation in young and old Niemann-Pick C1 model mice involves the alternative, M2, activation pathway and inhibition of macrophage apoptosis.Specific saposin C deficiency: CNS impairment and acid beta-glucosidase effects in the mouse Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration.Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin Common and uncommon pathogenic cascades in lysosomal storage diseases Deficiency of prion protein induces impaired autophagic flux in neurons.A marked paucity of granule cells in the developing cerebellum of the Npc1(-/-) mouse is corrected by a single injection of hydroxypropyl-β-cyclodextrin Oxidative stress in Niemann-Pick disease, type C Macroautophagy is not directly involved in the metabolism of amyloid precursor protein.Cholesterol homeostatic responses provide biomarkers for monitoring treatment for the neurodegenerative disease Niemann-Pick C1 (NPC1)Npc1 acting in neurons and glia is essential for the formation and maintenance of CNS myelin.Pathological roles of the VEGF/SphK pathway in Niemann-Pick type C neurons Anatomically defined neuron-based rescue of neurodegenerative Niemann-Pick type C disorder.Endosomal/lysosomal processing of gangliosides affects neuronal cholesterol sequestration in Niemann-Pick disease type C.β-Cyclodextrin-threaded biocleavable polyrotaxanes ameliorate impaired autophagic flux in Niemann-Pick type C disease.Temporal and cell-specific deletion establishes that neuronal Npc1 deficiency is sufficient to mediate neurodegeneration Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats.Neuronal loss of Drosophila NPC1a causes cholesterol aggregation and age-progressive neurodegeneration.Cholesterol accumulation is associated with lysosomal dysfunction and autophagic stress in Npc1 -/- mouse brain Heat Shock Protein Beta-1 Modifies Anterior to Posterior Purkinje Cell Vulnerability in a Mouse Model of Niemann-Pick Type C Disease Neuronal and epithelial cell rescue resolves chronic systemic inflammation in the lipid storage disorder Niemann-Pick C.Differential Transcriptome Networks between IDO1-Knockout and Wild-Type Mice in Brain Microglia and Macrophages.Ryanodine receptor antagonists adapt NPC1 proteostasis to ameliorate lipid storage in Niemann-Pick type C disease fibroblasts Astrocyte dysfunction triggers neurodegeneration in a lysosomal storage disorder.Autophagy and neuronal cell death in neurological disorders Quantitative proteomic analysis of Niemann-Pick disease, type C1 cerebellum identifies protein biomarkers and provides pathological insight.Complement is dispensable for neurodegeneration in Niemann-Pick disease type C.Sphingolipids: regulators of crosstalk between apoptosis and autophagy

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Cell-autonomous death of cerebellar purkinje neurons with autophagy in Niemann-Pick type C disease

http://www.wikidata.org/entity/Q24811579

description

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Cell-autonomous death of cereb ...... in Niemann-Pick type C disease

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Cell-autonomous death of cereb ...... in Niemann-Pick type C disease

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Cell-autonomous death of cereb ...... in Niemann-Pick type C disease

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Cell-autonomous death of cereb ...... in Niemann-Pick type C disease

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Cell-autonomous death of cereb ...... in Niemann-Pick type C disease

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Cell-autonomous death of cereb ...... in Niemann-Pick type C disease

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Dennis C Ko

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Hermogenes Manuel

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JoAnn Buchanan

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Ljiljana Milenkovic

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Matthew P Scott

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Steven M Beier

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Identification of HE1 as the second gene of Niemann-Pick C disease

Human Apg3p/Aut1p homologue is an authentic E2 enzyme for multiple substrates, GATE-16, GABARAP, and MAP-LC3, and facilitates the conjugation of hApg12p to hApg5p

HsAtg4B/HsApg4B/autophagin-1 cleaves the carboxyl termini of three human Atg8 homologues and delipidates microtubule-associated protein light chain 3- and GABAA receptor-associated protein-phospholipid conjugates

Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis

Acid sphingomyelinase deficient mice: a model of types A and B Niemann-Pick disease

Structure of a cholesterol-binding protein deficient in Niemann-Pick type C2 disease

LC3, a mammalian homologue of yeast Apg8p, is localized in autophagosome membranes after processing

Localization of Niemann-Pick C1 protein in astrocytes: implications for neuronal degeneration in Niemann- Pick type C disease

Combined analysis of oligonucleotide microarray data from transgenic and knockout mice identifies direct SREBP target genes

Transmembrane molecular pump activity of Niemann-Pick C1 protein

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