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51 Normann, Richard A.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
52 Digre, Kathleen B.; Couldwell, William T.; Amini, AminPhotophobia 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
53 Warner, Judith E. A.Intermittent reversal of Complete Ptosis-EganWarnerCASE REPORT: Ptosis secondary to dense oculomotor pareses generally improves over several months, but intermittent elevation of the eyelid has not been reported. The Author's describe two patients who demonstrated intermittent involuntary monocular eyelid elevation in an eye with complete ptosis cau...Eyelids; Ophthalmoplegia; Intraoperative Complications2006
54 Baehr, WolfgangCloning and molecular characterization of cGMP-gated ion channels from rod and cone photoreceptors of striped bass ( M. saxatilis ) retinaVertebrate photoreceptors respond to light with changes in membrane conductance that reflect the activity of cyclic-nucleotide gated channels (CNG channels). The functional features of these channels differ in rods and cones; to understand the basis of these differences we cloned CNG channels from t...Amino Acid Sequence; Computational Biology; Reverse Transcriptase Polymerase Chain Reaction2006
55 Zhang, KangMolecular and developmental characterization of the heat shock cognate 4 gene of Drosophila melanogasterThe Drosophila heat shock cognate gene 4 (hsc4), a member of the hsp70 gene family, encodes an abundant protein, hsc70, that is more similar to the constitutively expressed human protein than the Drosophila heat-inducible hsp70. Developmental expression revealed that hsc4 transcripts are enriched in...Embryo, Nonmammalian; Molecular Sequence Data1990
56 Baehr, WolfgangRetinoid 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
57 Baehr, Wolfgang; Zhang, KangElovl4 mRNA distribution in the developing mouse retina and phylogenetic conservation of Elovl4 genesPURPOSE: Stargardt-like macular dystrophy (STGD3) is an autosomal dominant form of early onset macular degeneration. The disease causing gene ELOVL4 encodes a protein that belongs to a family of proteins functioning in elongation of long chain fatty acids. The purpose of this study is to characteriz...Amino Acid Sequence; Cloning, Molecular; Molecular Sequence Data2003
58 Crockett, David K.Utility of gene-specific algorithms for predicting pathogenicity of uncertain gene variantsThe rapid advance of gene sequencing technologies has produced an unprecedented rate of discovery for genome variation in humans. A growing numbered of authoritative clinical repositories archive gene variants and disease phenotype, yet there are currently many more gene variants that lack clear ann...2012
59 Baehr, WolfgangNovel 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
60 Baehr, WolfgangReceptors for advanced glycation end products as progressive factors in age-related macular degenerationAdvanced glycation end products (AGE) exacerbate disease progression through two general mechanisms: modifying molecules and forming nondegradable aggregates, thus impairing normal cellular/tissue functions, and altering cellular function directly through receptor-mediated activation. In the prese...Advanced glycation end products; AGE; receptor for AGE-mediated cellular activation; RAGE2004
61 Bernstein, Paul S.; Gellermann, WernerRaman detection of macular carotenoid pigments in intact human retinaPURPOSE: To develop and test a novel noninvasive optical technique suitable for the objective measurement of macular carotenoid levels in human retina. METHODS: A resonance Raman scattering apparatus was constructed to measure carotenoid levels in flat-mounted human retinas and eyecups and in experi...Rana pipiens; Spectrum Analysis, Raman; Xanthophylls1998
62 Zhang, KangSpectrum and frequency of FZD4 mutations in familial exudative vitreoretinopathyMutations in the frizzled-4 gene (FZD4) have recently been associated with autosomal dominant familial exudative vitreoretinopathy (FEVR) in families linking to the EVR1 locus on the long arm of chromosome 11. The purpose of this study was to screen FZD4 in a panel of 40 patients with FEVR to ident...Frizzled-4 gene; FZD4; Mutation; autosomal dominant familial exudative autosomal dominant familial exudative; FEVR2004
63 Digre, Kathleen B.Serum vitamin A concentration is elevated in idiopathic intracranial hypertension.OBJECTIVE: The primary purpose was to investigate whether serum vitamin A concentration is associated with idiopathic intracranial hypertension (IIH). The secondary aim was to obtain pilot data regarding the amount of vitamin A ingested by patients and controls. BACKGROUND: Vitamin A is an attractiv...Vitamin A; Retinol; Idiopathic intracranial hypertension; Chromatography2007-02-01
64 Digre, Kathleen B.Selective MR imaging approach for evaluation of patients with Horner's syndrome.PURPOSE: To assess the usefulness of MR in the evaluation of patients with Horner's syndrome. PATIENTS AND METHODS: We prospectively performed MR imaging in 33 patients with Horner's syndrome (13 preganglionic and 20 postganglionic) using a protocol specifically designed for pre- and postganglionic ...Horner Syndrome; Magnetic Resonance Imaging; Epidemiology1992-01
65 Baehr, WolfgangIsolation and characterization of cGMP phosphodiesterase from bovine rod outer segmentsA simple purification method has been developed for isolation of bovine cGMP phosphodiesterase from photoreceptor rod outer segments. The enzyme is peripherally membrane bound in its native state and is present in relatively high concentrations. In the bovine photoreceptors its molar ratio to rhodop...Enzyme Activation; Macromolecular Substances; Trypsin/pharmacology1979
66 Normann, Richard A.Functional reorganization of primary visual cortex induced by electrical stimulation in the cat.Compared to the high degree of plasticity observed in a juvenile, mature sensory cortices have long been held to be immutable but, recently, researchers have suggested some plasticity persists in the mature cortex. Cortical reorganization has particular saliency to the development of a cortically ba...Cat; Electrophysiology; Phosphene; Plasticity; Receptive Field; Striate Cortex2005
67 Baehr, WolfgangPharmacological and rAAV gene therapy rescue of visual functions in a blind mouse model of Leber congenital amaurosisBACKGROUND: Leber congenital amaurosis (LCA), a heterogeneous early-onset retinal dystrophy, accounts for approximately 15% of inherited congenital blindness. One cause of LCA is loss of the enzyme lecithin:retinol acyl transferase (LRAT), which is required for regeneration of the visual photopigmen...Disease Models, Animal; Genetic Vectors; Molecular Sequence Data2005
68 Kriesel, John D.; Spruance, Spotswood L.Rocky Mountain spotted fever following cardiac transplantation.ROCKY MOUNTAIN SPOTTED FEVER was first recognized in the early 1 900s in the Snake River Valley of Idaho and the Bitterroot Valley of western Montana.t In 1909, Howard Taylor Ricketts established the ixodid tick as the vector for the disease.2 Following the tick bite, the incubation period aver...Male; Adult; Humans1993
69 Normann, Richard A.; Shoham, ShyEncoding 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
70 Digre, Kathleen B.; Skuster, Denise Z.Neurologic conditions presenting as psychiatric disorders.Understanding underlying neuroanatomic function helps physicians to localize defects and search for treatable neurologic conditions. Neurologic conditions such as Huntington's chorea, Wilson's disease, Gille de la Tourette syndrome, brain tumors, encephalitis and meningitis, neurodegenerative condit...Psychiatric Diagnosis; Neurologic Disease1992-06-15
71 Kriesel, John D.Miliary Tuberculosis and the postpartum stateIn 1985, a resurgence of tuberculosis began in the United States. In conjunction with this resurgence, there has been an increase in the number of atypical presentations of the disease. We recently treated a patient who had disseminated tuberculosis that became manifest in the postpartum state. Wher...Miliary tuberculosis; Postpartum1995
72 Olson, Randall J.Comparison of the effects of viscoelastic agents on clinical properties of the Unfolder lens injection systemPURPOSE: To evaluate the effect of 7 viscoelastic materials on the physical properties of the Unfolder lens injection system. SETTING: John A. Moran Eye Center, University of Utah School of Medicine, Salt Lake City, Utah, USA. METHODS: New sterile SI-40NB intraocular lenses (IOLs) were loaded into t...Lenses, Intraocular; Materials Testing; Hyaluronic Acid; Equipment Failure Analysis1999-07
73 Normann, Richard A.; Maynard, Edwin M.Neuronal interactions improve cortical population coding of movement directionInteractions among groups of neurons in primary motor cortex (MI) may convey information about motor behavior. We investigated the information carried by interactions in MI of macaque monkeys using a novel multielectrode array to record simultaneously from 12-16 neurons during an arm-reaching task. ...Motor Cortex; Monkey; Population Coding; Movement1999
74 Normann, Richard A.Effects of background illumination on the photoresponses of red and green cones1. The photoresponses of light- and dark-adapted red and green cone photoreceptors were recorded intracellularly in the retina of the turtle, Pseduemys scripta elegans. Background illumination produced similar effects on both types of cones. 2. In response to the onset of a prolonged, steady backgro...Turtles; Adaptation, Ocular; Retina; Signal Transmission1979
75 Kriesel, John D.; Baringer, J R; Spruance, Spotswood L.Correlation between detection of herpes simplex virus in oral secretions by PCR and susceptibility to experimental UV radiation-induced herpes labialis.We examined the oral secretions of 25 patients for herpes simplex virus (HSV) at the time of and following experimental UV radiation (UVR). HSV was detected in one or more oral secretion specimens in 5 of 12 (42%) cases by cell culture and in 8 of 12 (67%) cases by PCR. On the day of UVR, HSV was de...Polymerase Chain Reaction; Ultraviolet Rays; Simplexvirus1994
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