Impaired proteolysis underlies autophagic dysfunction in Niemann–Pick type C disease

Guidelines for the use and interpretation of assays for monitoring autophagy in higher eukaryotes

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Disruption and therapeutic rescue of autophagy in a human neuronal model of Niemann Pick type C1

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Sphingolipid Storage Affects Autophagic Metabolism of the Amyloid Precursor Protein and Promotes A  Generation

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Protein homeostasis and aging in neurodegeneration

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FYCO1 is a Rab7 effector that binds to LC3 and PI3P to mediate microtubule plus end–directed vesicle transport

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Reevaluation of Neurodegeneration in lurcher Mice: Constitutive Ion Fluxes Cause Cell Death with, Not by, Autophagy

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Endocytosis of beta-cyclodextrins is responsible for cholesterol reduction in Niemann-Pick type C mutant cells

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Autophagy in neurodegenerative disorders: pathogenic roles and therapeutic implications

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Increased Activity and Altered Subcellular Distribution of Lysosomal Enzymes Determine Neuronal Vulnerability in Niemann-Pick Type C1-Deficient Mice

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Chemical screen to reduce sterol accumulation in Niemann–Pick C disease cells identifies novel lysosomal acid lipase inhibitors

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Mechanisms and consequences of impaired lipid trafficking in Niemann–Pick type C1-deficient mammalian cells

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Structure of N-Terminal Domain of NPC1 Reveals Distinct Subdomains for Binding and Transfer of Cholesterol

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Conditional Niemann-Pick C mice demonstrate cell autonomous Purkinje cell neurodegeneration

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Constitutive Reactive Oxygen Species Generation from Autophagosome/Lysosome in Neuronal Oxidative Toxicity

(2009) Chisato Kubota et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Autophagy Inhibition Compromises Degradation of Ubiquitin-Proteasome Pathway Substrates

(2009) Viktor I. Korolchuk et al.   MOLECULAR CELL

Autophagy regulates lipid metabolism

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Chronic Cyclodextrin Treatment of Murine Niemann-Pick C Disease Ameliorates Neuronal Cholesterol and Glycosphingolipid Storage and Disease Progression

(2009) Cristin D. Davidson et al.   PLoS One

Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse

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Autophagy in the Pathogenesis of Disease

(2008) Beth Levine et al.   CELL

Tau deletion exacerbates the phenotype of Niemann–Pick type C mice and implicates autophagy in pathogenesis

(2008) Chris D. Pacheco et al.   HUMAN MOLECULAR GENETICS

Cholesterol Accumulation by Macrophages Impairs Phagosome Maturation

(2008) Kassidy K. Huynh et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Adapting Proteostasis for Disease Intervention

(2008) William E. Balch et al.   SCIENCE

The Itinerary of Autophagosomes: From Peripheral Formation to Kiss-and-Run Fusion with Lysosomes

(2008) Luca Jahreiss et al.   TRAFFIC