Impaired proteolysis underlies autophagic dysfunction in Niemann-Pick type C disease - Wikidata - wikimedia - TriplyDB

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

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

http://www.wikidata.org/entity/Q36714243

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Autophagy in the physiology and pathology of the central nervous system The lysosome: from waste bag to potential therapeutic target Early etiology of Alzheimer's disease: tipping the balance toward autophagy or endosomal dysfunction?Disruption in connexin-based communication is associated with intracellular Ca²⁺ signal alterations in astrocytes from Niemann-Pick type C mice It's a cell-eat-cell world: autophagy and phagocytosis.Mitochondria and quality control defects in a mouse model of Gaucher disease--links to Parkinson's disease SGPL1 (sphingosine phosphate lyase 1) modulates neuronal autophagy via phosphatidylethanolamine production.High sphingomyelin levels induce lysosomal damage and autophagy dysfunction in Niemann Pick disease type A.The pleiotropic roles of sphingolipid signaling in autophagy Leelamine mediates cancer cell death through inhibition of intracellular cholesterol transport.Defective macroautophagic turnover of brain lipids in the TgCRND8 Alzheimer mouse model: prevention by correcting lysosomal proteolytic deficits.α-Synuclein-independent histopathological and motor deficits in mice lacking the endolysosomal Parkinsonism protein Atp13a2.β-Cyclodextrin-threaded biocleavable polyrotaxanes ameliorate impaired autophagic flux in Niemann-Pick type C disease.Altered localization and functionality of TAR DNA Binding Protein 43 (TDP-43) in niemann- pick disease type C Autophagy flux in CA1 neurons of Alzheimer hippocampus: Increased induction overburdens failing lysosomes to propel neuritic dystrophy.Genetic and pharmacological evidence implicates cathepsins in Niemann-Pick C cerebellar degeneration.Necroptosis in Niemann-Pick disease, type C1: a potential therapeutic target.Lysosomal membrane permeability stimulates protein aggregate formation in neurons of a lysosomal disease WASH is required for lysosomal recycling and efficient autophagic and phagocytic digestion.Autophagy and Macropinocytosis: Keeping an Eye on the Corneal/Limbal Epithelia.Sphingolipid signalling mediates mitochondrial dysfunctions and reduced chronological lifespan in the yeast model of Niemann-Pick type C1.Emerging role of autophagy in pediatric neurodegenerative and neurometabolic diseases.Impaired autophagy in the lipid-storage disorder Niemann-Pick type C1 disease.Lysosomal membrane permeabilization in cell death: new evidence and implications for health and disease.Congenital disorders of autophagy: an emerging novel class of inborn errors of neuro-metabolism.Niemann-Pick type C2 deficiency impairs autophagy-lysosomal activity, mitochondrial function, and TLR signaling in adipocytes.Lysosome and endoplasmic reticulum quality control pathways in Niemann-Pick type C disease.Exploring the power of yeast to model aging and age-related neurodegenerative disorders.Modulating ryanodine receptors with dantrolene attenuates neuronopathic phenotype in Gaucher disease mice.Genetic and chemical correction of cholesterol accumulation and impaired autophagy in hepatic and neural cells derived from Niemann-Pick Type C patient-specific iPS cells.Cholesterol lowering effects of mono-lactose-appended β-cyclodextrin in Niemann-Pick type C disease-like HepG2 cells.Mitochondrial Dysfunction in Lysosomal Storage Disorders.Lysosomal pH-inducible supramolecular dissociation of polyrotaxanes possessing acid-labile N-triphenylmethyl end groups and their therapeutic potential for Niemann-Pick type C disease Niemann-Pick disease type C: introduction and main clinical features Quantitative Analysis of the Proteome Response to the Histone Deacetylase Inhibitor (HDACi) Vorinostat in Niemann-Pick Type C1 disease.Lysosomal and Mitochondrial Liaisons in Niemann-Pick Disease.L-leucine and SPNS1 coordinately ameliorate dysfunction of autophagy in mouse and human Niemann-Pick type C disease.LC3 Immunostaining in the Inferior Olivary Nuclei of Cats With Niemann-Pick Disease Type C1 Is Associated With Patterned Purkinje Cell Loss.Effects of cholesterol transport inhibitor U18666A on APP metabolism in rat primary astrocytes.Lipidomic and Transcriptomic Basis of Lysosomal Dysfunction in Progranulin Deficiency.

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

http://www.wikidata.org/entity/Q36714243

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2012 nî lūn-bûn

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2012年の論文

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2012年論文

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2012年論文

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2012年論文

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2012年论文

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2012年论文

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2012年论文

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name

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

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

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type

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

@ast

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

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prefLabel

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

@ast

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

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Human Molecular Genetics

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

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P2093

Andrew P Lieberman

http://www.w3.org/2001/XMLSchema#string

Chan Chung

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Matthew J Elrick

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Ting Yu

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P2860

Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression

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

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

Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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

p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy

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

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

Lysosomal cysteine proteases: facts and opportunities.

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

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4876-4887

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10.1093/HMG/DDS324

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22

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21

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3607480

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