Published in J Biol Chem on December 10, 2009
Ebola virus entry requires the cholesterol transporter Niemann-Pick C1. Nature (2011) 5.68
The cell biology of disease: lysosomal storage disorders: the cellular impact of lysosomal dysfunction. J Cell Biol (2012) 1.93
Normalizing glycosphingolipids restores function in CD4+ T cells from lupus patients. J Clin Invest (2014) 1.60
Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment. J Neurosci (2011) 1.33
Complex lipid trafficking in Niemann-Pick disease type C. J Inherit Metab Dis (2014) 1.20
Cathepsin B & L are not required for ebola virus replication. PLoS Negl Trop Dis (2012) 1.02
Niemann-Pick C disease and mobilization of lysosomal cholesterol by cyclodextrin. J Lipid Res (2014) 0.96
Potential vaccines and post-exposure treatments for filovirus infections. Viruses (2012) 0.94
Loss of Niemann-Pick C1 or C2 protein results in similar biochemical changes suggesting that these proteins function in a common lysosomal pathway. PLoS One (2011) 0.88
All-trans retinoic acid-triggered antimicrobial activity against Mycobacterium tuberculosis is dependent on NPC2. J Immunol (2014) 0.88
Sensitivity to lysosome-dependent cell death is directly regulated by lysosomal cholesterol content. PLoS One (2012) 0.88
Protein replacement therapy partially corrects the cholesterol-storage phenotype in a mouse model of Niemann-Pick type C2 disease. PLoS One (2011) 0.86
Niemann-Pick Type C2 protein contributes to the transport of endosomal cholesterol to mitochondria without interacting with NPC1. J Lipid Res (2012) 0.85
Loss of AP-5 results in accumulation of aberrant endolysosomes: defining a new type of lysosomal storage disease. Hum Mol Genet (2015) 0.85
Molecular pathways for intracellular cholesterol accumulation: common pathogenic mechanisms in Niemann-Pick disease Type C and cystic fibrosis. Arch Biochem Biophys (2011) 0.83
Somatic cell plasticity and Niemann-Pick type C2 protein: fibroblast activation. J Biol Chem (2010) 0.82
Multiple Surface Regions on the Niemann-Pick C2 Protein Facilitate Intracellular Cholesterol Transport. J Biol Chem (2015) 0.81
The Niemann-Pick C1 Inhibitor NP3.47 Enhances Gene Silencing Potency of Lipid Nanoparticles Containing siRNA. Mol Ther (2016) 0.77
Characteristics of highly polymorphic segmental copy-number variations observed in Japanese by BAC-array-CGH. J Biomed Biotechnol (2010) 0.77
APP overexpression in the absence of NPC1 exacerbates metabolism of amyloidogenic proteins of Alzheimer's disease. Hum Mol Genet (2015) 0.75
Commentary. Lysosomotropic agents. Biochem Pharmacol (1974) 12.28
Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis. Science (1997) 6.66
Identification of HE1 as the second gene of Niemann-Pick C disease. Science (2000) 5.08
Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium. Nat Med (2008) 3.86
Niemann-Pick disease type C. Clin Genet (2003) 3.64
Lysosomes behave as Ca2+-regulated exocytic vesicles in fibroblasts and epithelial cells. J Cell Biol (1997) 3.31
Rab proteins mediate Golgi transport of caveola-internalized glycosphingolipids and correct lipid trafficking in Niemann-Pick C cells. J Clin Invest (2002) 3.13
Visualization of Rab9-mediated vesicle transport from endosomes to the trans-Golgi in living cells. J Cell Biol (2002) 3.03
NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomes. Proc Natl Acad Sci U S A (2008) 2.81
Immunocytochemical characterization of the endocytic and phagolysosomal compartments in peritoneal macrophages. J Cell Biol (1992) 2.71
Repair of injured plasma membrane by rapid Ca2+-dependent endocytosis. J Cell Biol (2008) 2.66
The role of intraorganellar Ca(2+) in late endosome-lysosome heterotypic fusion and in the reformation of lysosomes from hybrid organelles. J Cell Biol (2000) 2.46
The Niemann-Pick C1 protein resides in a vesicular compartment linked to retrograde transport of multiple lysosomal cargo. J Biol Chem (1999) 2.45
Genetic evidence for nonredundant functional cooperativity between NPC1 and NPC2 in lipid transport. Proc Natl Acad Sci U S A (2004) 2.32
Abnormal transport along the lysosomal pathway in mucolipidosis, type IV disease. Proc Natl Acad Sci U S A (1998) 2.18
Fusion of lysosomes with late endosomes produces a hybrid organelle of intermediate density and is NSF dependent. J Cell Biol (1998) 2.12
Dense core lysosomes can fuse with late endosomes and are re-formed from the resultant hybrid organelles. J Cell Sci (1997) 2.10
Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations. J Neuropathol Exp Neurol (2001) 2.01
The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts. J Cell Biol (1989) 1.98
Dynamic movements of organelles containing Niemann-Pick C1 protein: NPC1 involvement in late endocytic events. Mol Biol Cell (2001) 1.87
Lysosome-endosome fusion and lysosome biogenesis. J Cell Sci (2000) 1.82
Replacement of endogenous cholesteryl esters of low density lipoprotein with exogenous cholesteryl linoleate. Reconstitution of a biologically active lipoprotein particle. J Biol Chem (1978) 1.71
Group C Niemann-Pick disease: faulty regulation of low-density lipoprotein uptake and cholesterol storage in cultured fibroblasts. FASEB J (1987) 1.71
Niemann-pick type C1 (NPC1) overexpression alters cellular cholesterol homeostasis. J Biol Chem (2000) 1.60
Repairing a torn cell surface: make way, lysosomes to the rescue. J Cell Sci (2002) 1.48
Cessation of rapid late endosomal tubulovesicular trafficking in Niemann-Pick type C1 disease. Proc Natl Acad Sci U S A (2001) 1.37
Evidence for retrograde traffic between terminal lysosomes and the prelysosomal/late endosome compartment. J Cell Sci (1994) 1.33
The sterol-sensing domain of the Niemann-Pick C1 (NPC1) protein regulates trafficking of low density lipoprotein cholesterol. J Biol Chem (2005) 1.33
Cholesterol accumulation sequesters Rab9 and disrupts late endosome function in NPC1-deficient cells. J Biol Chem (2006) 1.31
Intracellular trafficking of Niemann-Pick C proteins 1 and 2: obligate components of subcellular lipid transport. Biochim Biophys Acta (2004) 1.25
Neurotoxicity of reactive aldehydes: the concept of "aldehyde load" as demonstrated by neuroprotection with hydroxylamines. Brain Res (2006) 1.22
Telomerase immortalization upregulates Rab9 expression and restores LDL cholesterol egress from Niemann-Pick C1 late endosomes. J Lipid Res (2002) 1.22
Deficiency of niemann-pick type C-1 protein impairs release of human immunodeficiency virus type 1 and results in Gag accumulation in late endosomal/lysosomal compartments. J Virol (2009) 1.18
Brain polyamine levels are altered in Alzheimer's disease. Neurosci Lett (1995) 1.04
Membrane traffic to and from lysosomes. Biochem Soc Symp (2005) 1.03
NPC1/NPC2 function as a tag team duo to mobilize cholesterol. Proc Natl Acad Sci U S A (2008) 1.00
Niemann-Pick C1 functions in regulating lysosomal amine content. J Biol Chem (2008) 0.98
Structural and physical changes in lysosomes from isolated rat hepatocytes treated with methylamine. Biochim Biophys Acta (1983) 0.97
Cellular and lysosomal uptake of methylamine in isolated rat hepatocytes. Biochem J (1983) 0.94
Niemann-Pick C1 protein facilitates the efflux of the anticancer drug daunorubicin from cells according to a novel vesicle-mediated pathway. J Pharmacol Exp Ther (2005) 0.93
Massive cell vacuolization induced by organic amines such as procainamide. J Pharmacol Exp Ther (2004) 0.93
Ganglioside storage, hexosaminidase lability, and urinary oligosaccharides in adult Sandhoff's disease. Neurology (1987) 0.92
Regional and temporal alterations of ODC/polyamine system during ALS-like neurodegenerative motor syndrome in G93A transgenic mice. Neurochem Int (2005) 0.82
[THE QUANTITATIVE DETERMINATION OF GANGLIOSIDES AND THEIR NEURAMINIC ACID-FREE DERIVATIVES IN INFANTILE, JUVENILE AND FORMS OF AMAUROTIC IDIOCY AND AN UNUSUAL LATE INFANTILE BIOCHEMICAL FORM]. J Neurochem (1965) 0.82
A fluorescence resonance energy transfer-based approach for investigating late endosome-lysosome retrograde fusion events. Anal Biochem (2008) 0.81
A new approach for enhancing differential selectivity of drugs to cancer cells. ACS Chem Biol (2006) 1.62
Separate roles for the Golgi apparatus and lysosomes in the sequestration of drugs in the multidrug-resistant human leukemic cell line HL-60. J Biol Chem (2003) 1.53
The biosynthesis of anticoagulant heparan sulfate by the heparan sulfate 3-O-sulfotransferase isoform 5. Biochim Biophys Acta (2004) 1.14
Weak base permeability characteristics influence the intracellular sequestration site in the multidrug-resistant human leukemic cell line HL-60. J Biol Chem (2004) 1.12
Lysosomal sequestration of amine-containing drugs: analysis and therapeutic implications. J Pharm Sci (2007) 1.11
A novel assay reveals that weakly basic model compounds concentrate in lysosomes to an extent greater than pH-partitioning theory would predict. Mol Pharm (2005) 1.04
Mechanisms of amine accumulation in, and egress from, lysosomes. Bioanalysis (2009) 1.03
Uptake, distribution and diffusivity of reactive fluorophores in cells: implications toward target identification. Mol Pharm (2010) 1.00
Intracellular drug sequestration events associated with the emergence of multidrug resistance: a mechanistic review. Front Biosci (2005) 1.00
Niemann-Pick C1 functions in regulating lysosomal amine content. J Biol Chem (2008) 0.98
Self-assembly is important for TIP47 function in mannose 6-phosphate receptor transport. Traffic (2003) 0.97
Cationic amphiphilic drugs cause a marked expansion of apparent lysosomal volume: implications for an intracellular distribution-based drug interaction. Mol Pharm (2012) 0.95
Niemann-Pick C1 protein facilitates the efflux of the anticancer drug daunorubicin from cells according to a novel vesicle-mediated pathway. J Pharmacol Exp Ther (2005) 0.93
The role of lysosomes in limiting drug toxicity in mice. J Pharmacol Exp Ther (2010) 0.91
A chemical strategy to manipulate the intracellular localization of drugs in resistant cancer cells. Biochemistry (2005) 0.87
Mechanism of internalization of an ICAM-1-derived peptide by human leukemic cell line HL-60: influence of physicochemical properties on targeted drug delivery. Mol Pharm (2007) 0.86
A cell fractionation approach for the quantitative analysis of subcellular drug disposition. Pharm Res (2004) 0.84
Evaluating the roles of autophagy and lysosomal trafficking defects in intracellular distribution-based drug-drug interactions involving lysosomes. J Pharm Sci (2013) 0.84
cIBR effectively targets nanoparticles to LFA-1 on acute lymphoblastic T cells. Mol Pharm (2010) 0.84
Lysosomes contribute to anomalous pharmacokinetic behavior of melanocortin-4 receptor agonists. Pharm Res (2007) 0.82
Time-dependent effects of hydrophobic amine-containing drugs on lysosome structure and biogenesis in cultured human fibroblasts. J Pharm Sci (2014) 0.81
Effect of modification of the physicochemical properties of ICAM-1-derived peptides on internalization and intracellular distribution in the human leukemic cell line HL-60. Mol Pharm (2009) 0.80
Assessment of golgi apparatus versus plasma membrane-localized multi-drug resistance-associated protein 1. Mol Pharm (2008) 0.80
Lysosomotropic properties of weakly basic anticancer agents promote cancer cell selectivity in vitro. PLoS One (2012) 0.80
Drug-drug interactions involving lysosomes: mechanisms and potential clinical implications. Expert Opin Drug Metab Toxicol (2012) 0.79
Exposure of cells to hydrogen peroxide can increase the intracellular accumulation of drugs. Mol Pharm (2007) 0.79
Intracellular Distribution-based Anticancer Drug Targeting: Exploiting a Lysosomal Acidification Defect Associated with Cancer Cells. Mol Cell Pharmacol (2010) 0.78
Amine-containing molecules and the induction of an expanded lysosomal volume phenotype: a structure-activity relationship study. J Pharm Sci (2014) 0.78