PubRank

Steven J Fliesler

Author PubWeight™ 52.78‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 Retention of function without normal disc morphogenesis occurs in cone but not rod photoreceptors. J Cell Biol 2006 1.70
2 Intraretinal lipid transport is dependent on high density lipoprotein-like particles and class B scavenger receptors. Mol Vis 2006 1.49
3 The R172W mutation in peripherin/rds causes a cone-rod dystrophy in transgenic mice. Hum Mol Genet 2004 1.48
4 Long-term and age-dependent restoration of visual function in a mouse model of CNGB3-associated achromatopsia following gene therapy. Hum Mol Genet 2011 1.47
5 Uptake of cholesterol by the retina occurs primarily via a low density lipoprotein receptor-mediated process. Mol Vis 2006 1.47
6 A partial structural and functional rescue of a retinitis pigmentosa model with compacted DNA nanoparticles. PLoS One 2009 1.37
7 Modulating expression of peripherin/rds in transgenic mice: critical levels and the effect of overexpression. Invest Ophthalmol Vis Sci 2004 1.32
8 Impaired cone function and cone degeneration resulting from CNGB3 deficiency: down-regulation of CNGA3 biosynthesis as a potential mechanism. Hum Mol Genet 2009 1.30
9 The Cys214-->Ser mutation in peripherin/rds causes a loss-of-function phenotype in transgenic mice. Biochem J 2005 1.28
10 Gene delivery to mitotic and postmitotic photoreceptors via compacted DNA nanoparticles results in improved phenotype in a mouse model of retinitis pigmentosa. FASEB J 2009 1.22
11 Effect of Rds abundance on cone outer segment morphogenesis, photoreceptor gene expression, and outer limiting membrane integrity. J Comp Neurol 2007 1.20
12 Cholesterol suppresses cellular TGF-beta responsiveness: implications in atherogenesis. J Cell Sci 2007 1.20
13 Lipid hydroperoxide formation in the retina: correlation with retinal degeneration and light damage in a rat model of Smith-Lemli-Opitz syndrome. Exp Eye Res 2005 1.16
14 Differential requirements for retinal degeneration slow intermolecular disulfide-linked oligomerization in rods versus cones. Hum Mol Genet 2008 1.15
15 Formation of 7-dehydrocholesterol-containing membrane rafts in vitro and in vivo, with relevance to the Smith-Lemli-Opitz syndrome. J Lipid Res 2003 1.14
16 Outer segment oligomerization of Rds: evidence from mouse models and subcellular fractionation. Biochemistry 2008 1.12
17 Development and characterization of a hypomorphic Smith-Lemli-Opitz syndrome mouse model and efficacy of simvastatin therapy. Hum Mol Genet 2006 1.11
18 Lack of protein-tyrosine sulfation disrupts photoreceptor outer segment morphogenesis, retinal function and retinal anatomy. Eur J Neurosci 2010 1.08
19 Evidence for a circadian rhythm of susceptibility to retinal light damage. Photochem Photobiol 2002 1.06
20 Lipidomic analysis of the retina in a rat model of Smith-Lemli-Opitz syndrome: alterations in docosahexaenoic acid content of phospholipid molecular species. J Neurochem 2007 1.06
21 G-protein betagamma-complex is crucial for efficient signal amplification in vision. J Neurosci 2011 1.05
22 Light-induced exacerbation of retinal degeneration in a rat model of Smith-Lemli-Opitz syndrome. Exp Eye Res 2005 1.03
23 Alterations in retinal rod outer segment fatty acids and light-damage susceptibility in P23H rats. Mol Vis 2002 1.03
24 Retinal abnormalities associated with the G90D mutation in opsin. J Comp Neurol 2004 1.00
25 Deletion of the p85alpha regulatory subunit of phosphoinositide 3-kinase in cone photoreceptor cells results in cone photoreceptor degeneration. Invest Ophthalmol Vis Sci 2011 1.00
26 Cholesterol-dependent association of caveolin-1 with the transducin alpha subunit in bovine photoreceptor rod outer segments: disruption by cyclodextrin and guanosine 5'-O-(3-thiotriphosphate). Biochemistry 2003 0.99
27 Novel oxysterols observed in tissues and fluids of AY9944-treated rats: a model for Smith-Lemli-Opitz syndrome. J Lipid Res 2011 0.99
28 Early-onset, slow progression of cone photoreceptor dysfunction and degeneration in CNG channel subunit CNGB3 deficiency. Invest Ophthalmol Vis Sci 2011 0.98
29 7-Dehydrocholesterol-derived oxysterols and retinal degeneration in a rat model of Smith-Lemli-Opitz syndrome. Biochim Biophys Acta 2012 0.96
30 Onecut1 is essential for horizontal cell genesis and retinal integrity. J Neurosci 2013 0.95
31 Loss of caveolin-1 impairs retinal function due to disturbance of subretinal microenvironment. J Biol Chem 2012 0.93
32 Ion-current-based proteomic profiling of the retina in a rat model of Smith-Lemli-Opitz syndrome. Mol Cell Proteomics 2013 0.93
33 A comparison of the behavior of cholesterol and selected derivatives in mixed sterol-phospholipid Langmuir monolayers: a fluorescence microscopy study. Chem Phys Lipids 2005 0.93
34 Genetic supplementation of RDS alleviates a loss-of-function phenotype in C214S model of retinitis pigmentosa. Adv Exp Med Biol 2008 0.90
35 Late-onset cone photoreceptor degeneration induced by R172W mutation in Rds and partial rescue by gene supplementation. Invest Ophthalmol Vis Sci 2007 0.90
36 Rhodopsin: the functional significance of asn-linked glycosylation and other post-translational modifications. Ophthalmic Genet 2009 0.90
37 Photodamage generates 7-keto- and 7-hydroxycholesterol in the rat retina via a free radical-mediated mechanism. Photochem Photobiol 2009 0.89
38 Peroxisome deficiency causes a complex phenotype because of hepatic SREBP/Insig dysregulation associated with endoplasmic reticulum stress. J Biol Chem 2008 0.89
39 Disturbed cholesterol homeostasis in a peroxisome-deficient PEX2 knockout mouse model. Mol Cell Biol 2004 0.88
40 Differential distribution of proteins and lipids in detergent-resistant and detergent-soluble domains in rod outer segment plasma membranes and disks. J Neurochem 2007 0.88
41 Alteration of retinal rod outer segment membrane fluidity in a rat model of Smith-Lemli-Opitz syndrome. J Lipid Res 2008 0.88
42 Lipid differences in rod outer segment membranes of rats with P23H and S334ter opsin mutations. Mol Vis 2005 0.84
43 Differential developmental deficits in retinal function in the absence of either protein tyrosine sulfotransferase-1 or -2. PLoS One 2012 0.83
44 CNGA3 deficiency affects cone synaptic terminal structure and function and leads to secondary rod dysfunction and degeneration. Invest Ophthalmol Vis Sci 2012 0.81
45 Cfh genotype interacts with dietary glycemic index to modulate age-related macular degeneration-like features in mice. Invest Ophthalmol Vis Sci 2014 0.80
46 Peroxisome deficiency-induced ER stress and SREBP-2 pathway activation in the liver of newborn PEX2 knock-out mice. Biochim Biophys Acta 2012 0.80
47 Enzyme blockade: a nonradioactive method to determine the absolute rate of cholesterol synthesis in the brain. J Lipid Res 2004 0.79
48 Hepatic isoprenoid metabolism in a rat model of Smith-Lemli-Opitz Syndrome. Lipids 2013 0.79
49 The function of oligomerization-incompetent RDS in rods. Adv Exp Med Biol 2010 0.78
50 Lipids and lipid metabolism in the eye. J Lipid Res 2010 0.75
51 A comparison of the packing behavior of egg phosphatidylcholine with cholesterol and biogenically related sterols in Langmuir monolayer films. Chem Phys Lipids 2009 0.75