Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium.
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Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progressionNiemann-Pick disease type CLeucine-rich repeat kinase 2 regulates autophagy through a calcium-dependent pathway involving NAADPInteraction of AnxA6 with isolated and artificial lipid microdomains; importance of lipid composition and calcium contentEbola virus entry requires the cholesterol transporter Niemann-Pick C1The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunctionCholesterol trafficking is required for mTOR activation in endothelial cellsTPC2 is a novel NAADP-sensitive Ca2+ release channel, operating as a dual sensor of luminal pH and Ca2+The formation and function of ER-endosome membrane contact sitesDiagnostic workup and management of patients with suspected Niemann-Pick type C diseaseTrafficking and Functions of Bioactive Sphingolipids: Lessons from Cells and Model MembranesMolecular pathways for intracellular cholesterol accumulation: common pathogenic mechanisms in Niemann-Pick disease Type C and cystic fibrosisGenetic and laboratory diagnostic approach in Niemann Pick disease type CLysosomal storage diseases and the heat shock response: convergences and therapeutic opportunitiesGenetic dissection of a cell-autonomous neurodegenerative disorder: lessons learned from mouse models of Niemann-Pick disease type CThe lysosome: from waste bag to potential therapeutic targetFingolimod for the treatment of neurological diseases-state of play and future perspectivesUpstream deregulation of calcium signaling in Parkinson's diseaseEarly etiology of Alzheimer's disease: tipping the balance toward autophagy or endosomal dysfunction?Identification of dietary alanine toxicity and trafficking dysfunction in a Drosophila model of hereditary sensory and autonomic neuropathy type 1.Ca2+/H+ exchange by acidic organelles regulates cell migration in vivo.Disruption in connexin-based communication is associated with intracellular Ca²⁺ signal alterations in astrocytes from Niemann-Pick type C miceInhomogeneity Based Characterization of Distribution Patterns on the Plasma MembraneA C-terminally truncated mouse Best3 splice variant targets and alters the ion balance in lysosome-endosome hybrids and the endoplasmic reticulum.Lysosome and calcium dysregulation in Alzheimer's disease: partners in crimeThe GARP complex is required for cellular sphingolipid homeostasis.Reconstituted human TPC1 is a proton-permeable ion channel and is activated by NAADP or Ca2+High susceptibility to fatty liver disease in two-pore channel 2-deficient miceMLN64 mediates egress of cholesterol from endosomes to mitochondria in the absence of functional Niemann-Pick Type C1 proteinAcidic calcium stores open for business: expanding the potential for intracellular Ca2+ signalingBidirectional Ca²⁺ signaling occurs between the endoplasmic reticulum and acidic organellesDeveloping therapeutic approaches for metachromatic leukodystrophyTwo-pore channels (TPCs): current controversiesPre-symptomatic activation of antioxidant responses and alterations in glucose and pyruvate metabolism in Niemann-Pick Type C1-deficient murine brainAlteration of gene expression profile in Niemann-Pick type C mice correlates with tissue damage and oxidative stressMultiple cationic amphiphiles induce a Niemann-Pick C phenotype and inhibit Ebola virus entry and infectionEndo-lysosomal dysfunction in human proximal tubular epithelial cells deficient for lysosomal cystine transporter cystinosinCurcumin Mitigates the Intracellular Lipid Deposit Induced by Antipsychotics In VitroDefective Cytochrome P450-Catalysed Drug Metabolism in Niemann-Pick Type C DiseaseA novel role for α-tocopherol transfer protein (α-TTP) in protecting against chloroquine toxicity
P2860
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P2860
Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium.
description
2008 nî lūn-bûn
@nan
2008 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2008 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2008年の論文
@ja
2008年論文
@yue
2008年論文
@zh-hant
2008年論文
@zh-hk
2008年論文
@zh-mo
2008年論文
@zh-tw
2008年论文
@wuu
name
Niemann-Pick disease type C1 i ...... gulation of lysosomal calcium.
@ast
Niemann-Pick disease type C1 i ...... gulation of lysosomal calcium.
@en
Niemann-Pick disease type C1 i ...... gulation of lysosomal calcium.
@nl
type
label
Niemann-Pick disease type C1 i ...... gulation of lysosomal calcium.
@ast
Niemann-Pick disease type C1 i ...... gulation of lysosomal calcium.
@en
Niemann-Pick disease type C1 i ...... gulation of lysosomal calcium.
@nl
prefLabel
Niemann-Pick disease type C1 i ...... gulation of lysosomal calcium.
@ast
Niemann-Pick disease type C1 i ...... gulation of lysosomal calcium.
@en
Niemann-Pick disease type C1 i ...... gulation of lysosomal calcium.
@nl
P2093
P356
P1433
P1476
Niemann-Pick disease type C1 i ...... gulation of lysosomal calcium.
@en
P2093
Anthony J Morgan
Daniel J Sillence
David A Smith
Edward H Schuchman
Elena Elliot-Smith
Emyr Lloyd-Evans
Grant C Churchill
Xingxuan He
P2888
P304
P356
10.1038/NM.1876
P407
P577
2008-10-26T00:00:00Z
P5875
P6179
1031641209