PubRank

Daniel S Ory

Author PubWeight™ 71.27‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Triglyceride accumulation protects against fatty acid-induced lipotoxicity. Proc Natl Acad Sci U S A 2003 6.46
2 miR-33 links SREBP-2 induction to repression of sterol transporters. Proc Natl Acad Sci U S A 2010 3.99
3 Disruption of endoplasmic reticulum structure and integrity in lipotoxic cell death. J Lipid Res 2006 3.64
4 Cholesterol oxidation products are sensitive and specific blood-based biomarkers for Niemann-Pick C1 disease. Sci Transl Med 2010 3.42
5 Chronic cyclodextrin treatment of murine Niemann-Pick C disease ameliorates neuronal cholesterol and glycosphingolipid storage and disease progression. PLoS One 2009 2.90
6 Transgenic expression of fatty acid transport protein 1 in the heart causes lipotoxic cardiomyopathy. Circ Res 2004 2.50
7 Small nucleolar RNAs U32a, U33, and U35a are critical mediators of metabolic stress. Cell Metab 2011 2.16
8 A sensitive and specific LC-MS/MS method for rapid diagnosis of Niemann-Pick C1 disease from human plasma. J Lipid Res 2011 1.76
9 A regulatory role for 1-acylglycerol-3-phosphate-O-acyltransferase 2 in adipocyte differentiation. J Biol Chem 2006 1.73
10 NPC1 and NPC2 regulate cellular cholesterol homeostasis through generation of low density lipoprotein cholesterol-derived oxysterols. J Biol Chem 2003 1.69
11 Pregnane X receptor (PXR) activation: a mechanism for neuroprotection in a mouse model of Niemann-Pick C disease. Proc Natl Acad Sci U S A 2006 1.62
12 A critical role for eukaryotic elongation factor 1A-1 in lipotoxic cell death. Mol Biol Cell 2005 1.55
13 Niemann-Pick C1 protects against atherosclerosis in mice via regulation of macrophage intracellular cholesterol trafficking. J Clin Invest 2008 1.51
14 Niemann-Pick type C1 I1061T mutant encodes a functional protein that is selected for endoplasmic reticulum-associated degradation due to protein misfolding. J Biol Chem 2008 1.45
15 Impaired cholesterol efflux in senescent macrophages promotes age-related macular degeneration. Cell Metab 2013 1.42
16 The sterol-sensing domain of the Niemann-Pick C1 (NPC1) protein regulates trafficking of low density lipoprotein cholesterol. J Biol Chem 2005 1.33
17 Niemann-Pick disease type C clinical database: cognitive and coordination deficits are early disease indicators. Orphanet J Rare Dis 2013 1.33
18 Effectors of rapid homeostatic responses of endoplasmic reticulum cholesterol and 3-hydroxy-3-methylglutaryl-CoA reductase. J Biol Chem 2007 1.30
19 Fatty acid transport protein 1 and long-chain acyl coenzyme A synthetase 1 interact in adipocytes. J Lipid Res 2005 1.28
20 Genetic screening for Niemann-Pick disease type C in adults with neurological and psychiatric symptoms: findings from the ZOOM study. Hum Mol Genet 2013 1.24
21 δ-Tocopherol reduces lipid accumulation in Niemann-Pick type C1 and Wolman cholesterol storage disorders. J Biol Chem 2012 1.23
22 Characterization of oxysterols by electrospray ionization tandem mass spectrometry after one-step derivatization with dimethylglycine. Rapid Commun Mass Spectrom 2007 1.21
23 Collaborative development of 2-hydroxypropyl-β-cyclodextrin for the treatment of Niemann-Pick type C1 disease. Curr Top Med Chem 2014 1.19
24 Side chain oxygenated cholesterol regulates cellular cholesterol homeostasis through direct sterol-membrane interactions. J Biol Chem 2008 1.17
25 The non-coding RNA gadd7 is a regulator of lipid-induced oxidative and endoplasmic reticulum stress. J Biol Chem 2009 1.13
26 Microarray expression analysis and identification of serum biomarkers for Niemann-Pick disease, type C1. Hum Mol Genet 2012 1.10
27 Palmitate and lipopolysaccharide trigger synergistic ceramide production in primary macrophages. J Biol Chem 2012 1.08
28 Identification of Niemann-Pick C1 disease biomarkers through sphingolipid profiling. J Lipid Res 2013 1.07
29 25-Hydroxycholesterol increases the availability of cholesterol in phospholipid membranes. Biophys J 2011 0.99
30 Plasma sphingolipids associated with chronic obstructive pulmonary disease phenotypes. Am J Respir Crit Care Med 2015 0.95
31 Relative acidic compartment volume as a lysosomal storage disorder-associated biomarker. J Clin Invest 2014 0.92
32 Bone marrow transplantation increases efficacy of central nervous system-directed enzyme replacement therapy in the murine model of globoid cell leukodystrophy. Mol Genet Metab 2012 0.90
33 Oxysterols as non-genomic regulators of cholesterol homeostasis. Trends Endocrinol Metab 2012 0.89
34 Oligomerization of the murine fatty acid transport protein 1. J Biol Chem 2003 0.89
35 Lysosomal acid lipase deficiency impairs regulation of ABCA1 gene and formation of high density lipoproteins in cholesteryl ester storage disease. J Biol Chem 2011 0.88
36 Psychosine, the cytotoxic sphingolipid that accumulates in globoid cell leukodystrophy, alters membrane architecture. J Lipid Res 2013 0.86
37 The structural basis of cholesterol accessibility in membranes. Biophys J 2013 0.86
38 Side-chain oxysterols: from cells to membranes to molecules. Biochim Biophys Acta 2011 0.83
39 SmD3 regulates intronic noncoding RNA biogenesis. Mol Cell Biol 2012 0.83
40 Efficacy of N-acetylcysteine in phenotypic suppression of mouse models of Niemann-Pick disease, type C1. Hum Mol Genet 2013 0.83
41 The steroidal analog GW707 activates the SREBP pathway through disruption of intracellular cholesterol trafficking. J Lipid Res 2003 0.82
42 Liver X receptor agonist modulation of cholesterol efflux in mice with intestine-specific deletion of microsomal triglyceride transfer protein. Arterioscler Thromb Vasc Biol 2012 0.80
43 Side-chain oxysterols modulate cholesterol accessibility through membrane remodeling. Biochemistry 2014 0.79
44 Box C/D small nucleolar RNA (snoRNA) U60 regulates intracellular cholesterol trafficking. J Biol Chem 2013 0.79
45 Development and validation of LC-MS/MS method for determination of very long acyl chain (C22:0 and C24:0) ceramides in human plasma. Anal Bioanal Chem 2013 0.79
46 Altered distribution and function of natural killer cells in murine and human Niemann-Pick disease type C1. Blood 2013 0.79
47 Tracking the subcellular fate of 20(s)-hydroxycholesterol with click chemistry reveals a transport pathway to the Golgi. J Biol Chem 2014 0.79
48 Altered transition metal homeostasis in Niemann-Pick disease, type C1. Metallomics 2013 0.76
49 Synthesis of the enantiomer of the oxysterol-antagonist LY295427. Steroids 2011 0.76
50 Rpl13a small nucleolar RNAs regulate systemic glucose metabolism. J Clin Invest 2016 0.75