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101 Loss of ER retention and sequestration of the wild-type ELOVL4 by Stargardt disease dominant negative mutantsPURPOSE: Mutations in ELOVL4, a member of the fatty acid elongase (ELO) family, are responsible for autosomal dominant Stargardt-like macular degeneration. The specific role of ELOVL4 in photoreceptors and the degenerative events induced by dominant ELOVL4 mutations are not well understood. As a fir...Macular Degeneration; Transcription Factor CHOP; Molecular Chaperones2005
102 Mobility performance with a pixelized vision systemA visual prosthesis, based on electrical stimulation of the visual cortex, has been suggested as a means for partially restoring functional vision in the blind. The prosthesis would create a pixelized visual sense consisting of punctate spots of light (phosphenes). The present study investigated the...Visual Prosthesis; Mobility; Phosphene Simulator1992
103 Sedimentation field-flow fractionation: a method for studying particulates in cataractous lensIt is shown that the technique of sedimentation field-flow fractionation (sedimentation [sed] FFF) can be used to determine the particle content and particle size distribution of normal and cataractous lenses. A 31-year-old normal human lens, for example, showed a particle content of 1.5% by weight ...Fractionation; Cataractous Lens; Particulates1984
104 Role for nitric oxide in the development of the ferret retinogeniculate projectionThe ferret retinogeniculate projection segregates into eye-specific layers during the first postnatal week and into ON/OFF sublaminae, which receive inputs from either on-center or off-center retinal ganglion cells, during the third and fourth postnatal weeks. The restriction of retinogeniculate axo...Enzyme Inhibitors; Visual Pathways; Wheat Germ Agglutinin-Horseradish Peroxidase Conjugate1996
105 Study of printed spiral coils for neuroprosthetic transcranial telemetry applicationsWe have explored the use of printed spiral coils (PSC's) for neuroprosthetic transcranial telemetry applications. We fabricated two-dimensional PSC's on a thin (25 microns) polyimide substrate using copper (35 microns) as a conducting material. All the coils had a fixed inner diameter of 1.0 cm. We ...Blindness; Neuroprosthesis; Transcranial Telemetry System; Prosthesis Design1998
106 Evaluation of the G protein coupled receptor-75 (GPR75) in age related macular degeneration2001
107 Biochemical characterization of the retinoid isomerase system of the eyeWe have previously shown that membranes from the retinal pigment epithelium can transform added all-trans-retinol into a mixture of 11-cis-retinoids, demonstrating the "missing reaction" in the visual cycle for the first time (Bernstein, P. S., Law, W. C., and Rando, R. R. (1987) Proc. Natl. Acad. S...Hydrogen-Ion Concentration1987
108 Update on a long-term, prospective study of capsulotomy and retinal detachment rates after cataract surgeryPURPOSE: To evaluate the retinal detachment risks and neodymium:YAG (Nd:YAG) capsulotomy rates associated with different cataract approaches and intraocular lens (IOL) styles in a long-term,prospective clinical study. SETTING: Clinical practice of 1 ophthalmologist, Fort Collins, Colorado, USA. METH...Cataract Extraction; Laser Therapy; Retinal Detachment; Risk Factors2000-07
109 Familial advanced sleep-phase syndrome: a short-period circadian rhythmvariant in humansBiological circadian clocks oscillate with an approximately 24-hour period, are ubiquitous, and presumably confer a selective advantage by anticipating the transitions between day and night. The circadian rhythms of sleep, melatonin secretion and body core temperature are thought to be generated by ...Activity Cycles; Matched-Pair Analysis; Polysomnography1999
110 Dysphotopsia in phakic and pseudophakic patients: incidence and relation to intraocular lens type(2)PURPOSE: To determine the relationship between various intraocular lens (IOL) types and the incidence of unwanted light images. SETTING: The Moran Eye Center, University of Utah, Salt Lake City, Utah, USA. METHODS: A telephone questionnaire was administered to 302 postoperative patients who had rece...Cataract Extraction; Visual Acuity; Lenses, Intraocular; Lens Implantation, Intraocular2000-06
111 Human interstitial retinoid-binding protein. Gene structure and primary structureInterstitial retinoid-binding protein (IRBP) is synthesized and secreted by rod photoreceptor cells into the interphotoreceptor matrix and is known to bind retinoids and fatty acids. We have used cDNA clones encoding human IRBP to isolate a 15-kilobase genomic fragment that encompasses the complete ...Molecular Sequence Data; Eye Proteins; Retinol-Binding Proteins; Messenger RNA1989-05-15
112 Molecular characterization of a third member of the guanylyl cyclase-activating protein subfamilyThe mammalian retina contains at least two guanylyl cyclases (GC1 and GC2) and two guanylyl cyclase-activating proteins (GCAP1 and GCAP2). Here we present evidence of the presence of a new photoreceptor-specific GCAP, termed GCAP3, which is closely related to GCAP1. The sequence similarity of GCAP3 ...Amino Acid Sequence; Cloning, Molecular; Gene Expression Regulation1999
113 Encoding mechanisms for sensory neurons studied with a multielectrode array in the cat dorsal root ganglionRecent advances in microelectrode array technology now permit a direct examination of the way populations of sensory neurons encode information about a limb's position in space. To address this issue, we recorded nerve impulses from about 100 single units simultaneously in the L6 and L7 dorsal root ...Sensory; Encoding; Multielectrode; Dorsal Root Ganglion; Cutaneour; Muscle2004
114 Retinoid absorption and storage is impaired in mice lacking lecithin:retinol acyltransferase (LRAT)Lecithin:retinol acyltransferase (LRAT) is believed to be the predominant if not the sole enzyme in the body responsible for the physiologic esterification of retinol. We have studied Lrat-deficient (Lrat-/-) mice to gain a better understanding of how these mice take up and store dietary retinoids a...Chromatography, High Pressure Liquid; Mice, Transgenic; Tissue Distribution2005
115 Identification and functional consequences of a new mutation (E155G) in the gene for GCAP1 that causes autosomal dominant cone dystrophy2001
116 Experimentally induced retinal projections to the ferret auditory thalamus: development of clustered eye-specific patterns in a novel targetWe have examined the relative role of afferents and targets in pattern formation using a novel preparation, in which retinal projections in ferrets are induced to innervate the medial geniculate nucleus (MGN). We find that retinal projections to the MGN are arranged in scattered clusters. Clusters a...Retina; Ferrets1997
117 Novel mutation (I143NT) in guanylate cyclase-activating protein 1 (GCAP1) associated with autosomal dominant cone degenerationPURPOSE: To identify pathogenic mutations in the guanylate cyclase-activating protein 1 (GCAP1) and GCAP2 genes and to characterize the biochemical effect of mutation on guanylate cyclase (GC) stimulation. METHODS: The GCAP1 and GCAP2 genes were screened by direct sequencing for mutations in 216 pat...DNA Mutational Analysis; Mutation, Missense; Electroretinography2004
118 Mechanism of reductive activation of potato tuber ADP-glucose pyrophosphorylase.The potato tuber (Solanum tuberosum L.) ADP-glucose pyrophosphorylase activity is activated by a incubation with ADP-glucose and dithiothreitol or by ATP, glucose- 1-phosphate, Ca2+, and dithiothreitol. The activation was accompanied by the appearance of new sulfhydryl groups as determined with 5, 5...Glucosephosphates; Enzyme Activation; Adenosine Triphosphate1998
119 Heterozygous and homozygous mutations in PITX3 in a large Lebanese family with posterior polar cataracts and neurodevelopmental abnormalitiesPURPOSE: The PITX3 gene, which codes for a homeobox bicoidlike transcription factor is responsible for dominant cataract and anterior segment mesenchymal dysgenesis in humans. In the current study, a family with autosomal dominant posterior polar cataract (PPC) and a PITX3 mutation that cosegregates...Chromosomes, Human, Pair 10; Homozygote; Lod Score2006
120 Clinical comparison of single-piece and three-piece truncated hydrophobic acrylic intraocular lensesPURPOSE: To determine the clinical differences between three-piece (3P) and single-piece (SP) truncated hydrophobic acrylic intraocular lenses (IOL). DESIGN: Retrospective cohort clinical study. METHODS: The setting was an academic clinical practice. The patient population consisted of subjects with...Lenses, Intraocular; Patient Satisfaction; Postoperative Complications; Prosthesis Design; Visual Acuity2003-10
121 Congenital anomalies of the optic nerve in one family.We present three cases of congenital optic disc anomalies in one family who underwent an extensive diagnostic workup to exclude any intracranial pathology. One patient had elevated optic nerve heads and was hospitalized for multiple diagnostic tests including a lumbar puncture and magnetic resonance...Optic Nerve; Congenital Anomalies1992-04-24
122 Analysis of the dynamic mutation in the SCA7 gene shows marked parental effects on CAG repeat transmission.The gene for spinocerebellar ataxia 7 (SCA7) includes a transcribed, translated CAG tract that is expanded in SCA7 patients. We have determined expansions in 73 individuals from 17 SCA7 kindreds and compared them with repeat lengths of 180 unaffected individuals. Subjects with abnormal expansions co...Genetics; Biosynthesis; SCA7 Gene1998-03-07
123 Coding of position by simultaneously recorded sensory neurones in the cat dorsal root ganglionMuscle, cutaneous and joint afferents continuously signal information about the position and movement of individual joints. How does the nervous system extract more global information, for example about the position of the foot in space? To study this question we used microelectrode arrays to record...Cats; Sensory Neurones; Movement; Limb Position2004
124 Control of retinal sensitivity. I. Light and dark adaptation of vertebrate rods and conesRods and cones in Necturus respond with graded hyperpolarization to test flashes spanning about 3.5 log units of intensity. Steady background levels hyperpolarize the rods, and the rod responses become progressively smaller as background level is increased. In cones, higher background levels reduce...Retina;Light and Dark Adaptation; Recepters; Bipolars; Ganglion Cells1974
125 Photophobia in a blind patient: An alternate visual pathway. Case report.Photophobia is a common neurological and ophthalmological symptom that has been associated with a growing number of neurosurgical conditions, especially compressive lesions. The exact signaling pathways and neurophysiological features of the disorder are not well understood; however, data from multi...Photophobia, Blindness, Signaling Pathway, Pretectal Nuclei, Trigeminal Pathway2006-12-14
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