Published in Mol Vis on December 30, 2005
Evaluation of the adeno-associated virus mediated long-term expression of channelrhodopsin-2 in the mouse retina. Mol Vis (2009) 1.13
KCNQ1 channels voltage dependence through a voltage-dependent binding of the S4-S5 linker to the pore domain. J Biol Chem (2010) 1.10
N-terminal truncated intracellular matrix metalloproteinase-2 induces cardiomyocyte hypertrophy, inflammation and systolic heart failure. PLoS One (2013) 0.94
Transduction of the inner mouse retina using AAVrh8 and AAVrh10 via intravitreal injection. Exp Eye Res (2010) 0.83
Identification of a therapeutic dose of continuously delivered erythropoietin in the eye using an inducible promoter system. Curr Gene Ther (2013) 0.82
Outer Plexiform Layer Structures Are Not Altered Following AAV-Mediated Gene Transfer in Healthy Rat Retina. Front Neurol (2017) 0.75
Effects of Repeated Anesthesia Containing Urethane on Tumor Formation and Health Scores in Male C57BL/6J Mice. J Am Assoc Lab Anim Sci (2016) 0.75
Safety and efficacy of gene transfer for Leber's congenital amaurosis. N Engl J Med (2008) 17.21
Age-dependent effects of RPE65 gene therapy for Leber's congenital amaurosis: a phase 1 dose-escalation trial. Lancet (2009) 6.71
Long-term restoration of rod and cone vision by single dose rAAV-mediated gene transfer to the retina in a canine model of childhood blindness. Mol Ther (2005) 4.74
A quantitative account of the activation steps involved in phototransduction in amphibian photoreceptors. J Physiol (1992) 4.10
Identifying photoreceptors in blind eyes caused by RPE65 mutations: Prerequisite for human gene therapy success. Proc Natl Acad Sci U S A (2005) 4.04
Gene therapy for Leber's congenital amaurosis is safe and effective through 1.5 years after vector administration. Mol Ther (2009) 3.93
Dark adaptation and the retinoid cycle of vision. Prog Retin Eye Res (2004) 3.87
Phototransduction in transgenic mice after targeted deletion of the rod transducin alpha -subunit. Proc Natl Acad Sci U S A (2000) 3.72
Reversal of blindness in animal models of leber congenital amaurosis using optimized AAV2-mediated gene transfer. Mol Ther (2008) 3.28
Massive light-driven translocation of transducin between the two major compartments of rod cells: a novel mechanism of light adaptation. Neuron (2002) 3.09
UV- and midwave-sensitive cone-driven retinal responses of the mouse: a possible phenotype for coexpression of cone photopigments. J Neurosci (1999) 2.79
Impairment of the transient pupillary light reflex in Rpe65(-/-) mice and humans with leber congenital amaurosis. Invest Ophthalmol Vis Sci (2004) 2.72
Small interfering RNA (siRNA) targeting VEGF effectively inhibits ocular neovascularization in a mouse model. Mol Vis (2003) 2.68
The control of phosphodiesterase in rod disk membranes: kinetics, possible mechanisms and significance for vision. Vision Res (1979) 2.65
In utero gene therapy rescues vision in a murine model of congenital blindness. Mol Ther (2004) 2.56
Cone-like morphological, molecular, and electrophysiological features of the photoreceptors of the Nrl knockout mouse. Invest Ophthalmol Vis Sci (2005) 2.51
Recovery phase of the murine rod photoresponse reconstructed from electroretinographic recordings. J Neurosci (1996) 2.50
Serotype-dependent packaging of large genes in adeno-associated viral vectors results in effective gene delivery in mice. J Clin Invest (2008) 2.40
Mutations in ABCA4 result in accumulation of lipofuscin before slowing of the retinoid cycle: a reappraisal of the human disease sequence. Hum Mol Genet (2004) 2.30
The light response of ON bipolar neurons requires G[alpha]o. J Neurosci (2000) 2.19
Lateral connectivity and contextual interactions in macaque primary visual cortex. Neuron (2002) 2.16
Safety of recombinant adeno-associated virus type 2-RPE65 vector delivered by ocular subretinal injection. Mol Ther (2006) 2.13
Lighting a candle in the dark: advances in genetics and gene therapy of recessive retinal dystrophies. J Clin Invest (2010) 2.13
Novel adeno-associated virus serotypes efficiently transduce murine photoreceptors. J Virol (2007) 2.05
Eye movement recordings as an effectiveness indicator of gene therapy in RPE65-deficient canines: implications for the ocular motor system. Invest Ophthalmol Vis Sci (2006) 2.01
Kinetics of recovery of the dark-adapted salamander rod photoresponse. J Gen Physiol (1998) 1.98
Analysis of ERG a-wave amplification and kinetics in terms of the G-protein cascade of phototransduction. Invest Ophthalmol Vis Sci (1994) 1.88
Three-year follow-up after unilateral subretinal delivery of adeno-associated virus in patients with Leber congenital Amaurosis type 2. Ophthalmology (2013) 1.85
Regulation of drug reward by cAMP response element-binding protein: evidence for two functionally distinct subregions of the ventral tegmental area. J Neurosci (2005) 1.84
The role of steady phosphodiesterase activity in the kinetics and sensitivity of the light-adapted salamander rod photoresponse. J Gen Physiol (2000) 1.84
Induction of antigen-specific CD4+ T-cell anergy and deletion by in vivo viral gene transfer. Blood (2004) 1.84
The gain of rod phototransduction: reconciliation of biochemical and electrophysiological measurements. Neuron (2000) 1.83
Novel AAV serotypes for improved ocular gene transfer. J Gene Med (2008) 1.80
Visual transduction in vertebrate rods and cones: a tale of two transmitters, calcium and cyclic GMP. Vision Res (1986) 1.70
ATP mediates rapid reversal of cyclic GMP phosphodiesterase activation in visual receptor membranes. Nature (1980) 1.67
Gene therapy for choroideremia: in vitro rescue mediated by recombinant adenovirus. Vision Res (2003) 1.63
AAV-mediated gene therapy for choroideremia: preclinical studies in personalized models. PLoS One (2013) 1.62
Mice lacking G-protein receptor kinase 1 have profoundly slowed recovery of cone-driven retinal responses. J Neurosci (2000) 1.57
Kinetics and components of the flash photocurrent of isolated retinal rods of the larval salamander, Ambystoma tigrinum. J Physiol (1987) 1.56
Dosage thresholds for AAV2 and AAV8 photoreceptor gene therapy in monkey. Sci Transl Med (2011) 1.54
Light damage induced changes in mouse retinal gene expression. Exp Eye Res (2004) 1.54
The human visual cortex responds to gene therapy-mediated recovery of retinal function. J Clin Invest (2011) 1.52
The Y99C mutation in guanylyl cyclase-activating protein 1 increases intracellular Ca2+ and causes photoreceptor degeneration in transgenic mice. J Neurosci (2004) 1.51
Quantification of the cytoplasmic spaces of living cells with EGFP reveals arrestin-EGFP to be in disequilibrium in dark adapted rod photoreceptors. J Cell Sci (2004) 1.47
Mutations in RPGR and RP2 account for 15% of males with simplex retinal degenerative disease. Invest Ophthalmol Vis Sci (2012) 1.47
The density and photosensitivity of human rhodopsin in the living retina. J Physiol (1974) 1.45
Retinal ganglion cell loss induced by acute optic neuritis in a relapsing model of multiple sclerosis. Mult Scler (2006) 1.44
Imaging light responses of targeted neuron populations in the rodent retina. J Neurosci (2011) 1.41
A photoreceptor-specific cadherin is essential for the structural integrity of the outer segment and for photoreceptor survival. Neuron (2001) 1.41
ABCA4-associated retinal degenerations spare structure and function of the human parapapillary retina. Invest Ophthalmol Vis Sci (2005) 1.40
Cyclic GMP can increase rod outer-segment light-sensitive current 10-fold without delay of excitation. Nature (1985) 1.40
Systemic but not intraocular Epo gene transfer protects the retina from light-and genetic-induced degeneration. Mol Ther (2004) 1.35
Safety and efficacy of subretinal readministration of a viral vector in large animals to treat congenital blindness. Sci Transl Med (2010) 1.35
Rod outer segment structure influences the apparent kinetic parameters of cyclic GMP phosphodiesterase. J Gen Physiol (1994) 1.32
Lenalidomide-induced upregulation of CD80 on tumor cells correlates with T-cell activation, the rapid onset of a cytokine release syndrome and leukemic cell clearance in chronic lymphocytic leukemia. Haematologica (2009) 1.31
A fluorescent, genetically-encoded voltage probe capable of resolving action potentials. PLoS One (2012) 1.31
Disruption of intraflagellar protein transport in photoreceptor cilia causes Leber congenital amaurosis in humans and mice. J Clin Invest (2011) 1.31
Nrl-knockout mice deficient in Rpe65 fail to synthesize 11-cis retinal and cone outer segments. Invest Ophthalmol Vis Sci (2008) 1.31
The kinetics of inactivation of the rod phototransduction cascade with constant Ca2+i. J Gen Physiol (1996) 1.29
Herpes simplex virus type 1 glycoprotein e is required for axonal localization of capsid, tegument, and membrane glycoproteins. J Virol (2005) 1.29
Tie2Cre-mediated inactivation of plexinD1 results in congenital heart, vascular and skeletal defects. Dev Biol (2008) 1.29
Functionally rodless mice: transgenic models for the investigation of cone function in retinal disease and therapy. Vision Res (2002) 1.28
RGS9-1 is required for normal inactivation of mouse cone phototransduction. Mol Vis (2001) 1.27
A theory of the pi1 and pi3 color mechanisms of Stiles. Vision Res (1979) 1.27
Systemic rapamycin inhibits retinal and choroidal neovascularization in mice. Mol Vis (2004) 1.27
Type 3 deiodinase, a thyroid-hormone-inactivating enzyme, controls survival and maturation of cone photoreceptors. J Neurosci (2010) 1.26
Diffusion coefficient of cyclic GMP in salamander rod outer segments estimated with two fluorescent probes. Biophys J (1993) 1.22
Progress in gene therapy for neurological disorders. Nat Rev Neurol (2013) 1.22
Dissecting the axoneme interactome: the mammalian orthologue of Chlamydomonas PF6 interacts with sperm-associated antigen 6, the mammalian orthologue of Chlamydomonas PF16. Mol Cell Proteomics (2005) 1.21
Cyclic GMP increases photocurrent and light sensitivity of retinal cones. Nature (1985) 1.21
Protons suppress the dark current of frog retinal rods. J Physiol (1984) 1.20
Rushton's paradox: rod dark adaptation after flash photolysis. J Physiol (1975) 1.18
Extreme responsiveness of the pupil of the dark-adapted mouse to steady retinal illumination. Invest Ophthalmol Vis Sci (1998) 1.17
Suppression and replacement gene therapy for autosomal dominant disease in a murine model of dominant retinitis pigmentosa. Mol Ther (2011) 1.16
In vivo delivery of recombinant viruses to the fetal murine cochlea: transduction characteristics and long-term effects on auditory function. Mol Ther (2006) 1.15
Rhodopsin flash photolysis in man. J Physiol (1975) 1.15
A comparison of Depodur, a novel, single-dose extended-release epidural morphine, with standard epidural morphine for pain relief after lower abdominal surgery. Anesth Analg (2005) 1.15
Deficiency of SPAG16L causes male infertility associated with impaired sperm motility. Biol Reprod (2005) 1.14
Lifetime regulation of G protein-effector complex: emerging importance of RGS proteins. Neuron (1998) 1.14
Oxygen distribution and vascular injury in the mouse eye measured by phosphorescence-lifetime imaging. Appl Opt (2005) 1.13
Increased expression of ceruloplasmin in the retina following photic injury. Mol Vis (2003) 1.13
The ocular albinism type 1 protein, an intracellular G protein-coupled receptor, regulates melanosome transport in pigment cells. Hum Mol Genet (2008) 1.12
Double cones as a basis for a new type of polarization vision in vertebrates. Nature (1991) 1.12
Steric volume exclusion sets soluble protein concentrations in photoreceptor sensory cilia. Proc Natl Acad Sci U S A (2011) 1.11
Fractionated subcutaneous rituximab is well-tolerated and preserves CD20 expression on tumor cells in patients with chronic lymphocytic leukemia. Haematologica (2009) 1.11
Impact of signaling microcompartment geometry on GPCR dynamics in live retinal photoreceptors. J Gen Physiol (2012) 1.10
The magnitude, time course and spatial distribution of current induced in salamander rods by cyclic guanine nucleotides. J Physiol (1990) 1.09
Long-term inducible gene expression in the eye via adeno-associated virus gene transfer in nonhuman primates. Hum Gene Ther (2005) 1.07
Pharmacological regulation of protein expression from adeno-associated viral vectors in the eye. Mol Ther (2002) 1.07
Accelerated mortality from hydrocephalus and pneumonia in mice with a combined deficiency of SPAG6 and SPAG16L reveals a functional interrelationship between the two central apparatus proteins. Cell Motil Cytoskeleton (2007) 1.07
Disease expression in Usher syndrome caused by VLGR1 gene mutation (USH2C) and comparison with USH2A phenotype. Invest Ophthalmol Vis Sci (2005) 1.06
The ocular albinism type 1 (OA1) G-protein-coupled receptor functions with MART-1 at early stages of melanogenesis to control melanosome identity and composition. Hum Mol Genet (2009) 1.06