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CreatorTitleDescriptionSubjectDate
126 Baehr, WolfgangNoninvasive two-photon imaging reveals retinyl ester storage structures in the eyeVisual sensation in vertebrates is triggered when light strikes retinal photoreceptor cells causing photoisomerization of the rhodopsin chromophore 11-cis-retinal to all-trans-retinal. The regeneration of preillumination conditions of the photoreceptor cells requires formation of 11-cis-retinal in t...Microscopy, Fluorescence; Visual Perception; Membrane Proteins2004
127 Bernstein, Paul S.; Gellermann, WernerMacular pigment Raman detector for clinical applicationsClinical studies of carotenoid macular pigments (MP) have been limited by the lack of noninvasive, objective instruments. We introduce a novel noninvasive optical instrument, an MP Raman detector, for assessment of the carotenoid status of the human retina in vivo. The instrument uses resonant excit...Diagnosis, Computer-Assisted; Equipment Failure Analysis; Sensitivity and Specificity2004
128 Baehr, WolfgangLecithin-retinol acyltransferase is essential for accumulation of all-trans-retinyl esters in the eye and in the liverLecithin-retinol acyltransferase (LRAT), an enzyme present mainly in the retinal pigmented epithelial cells and liver, converts all-trans-retinol into all-trans-retinyl esters. In the retinal pigmented epithelium, LRAT plays a key role in the retinoid cycle, a two-cell recycling system that replenis...Antibodies, Monoclonal; Chromatography, High Pressure Liquid; Microscopy, Fluorescence2004
129 Olson, Randall J.Early experiences with microphacoMicrophaco is the separation of irrigation from aspiration that allows removal of cataracts through sub-2mm incisions. While proven intraocular lenses (IOLs) are not available at this time to take advantage of the small wounds, these wounds are advantageous from a separating irrigation standpoint w...Microphaco; Cataracts; Irrigation; Wounds2004
130 Zhang, KangNovel mutation in the ELOVL4 gene causes autosomal dominant Stargardt-like macular dystrophyPURPOSE. To conduct clinical and genetic studies in a European family with autosomal dominant Stargardt-like macular dystrophy (adSTGD-like MD) and to investigate the functional consequences of a novel ELOVL4 mutationMacular dystrophy; ELOVL4 mutation; Genetics2004
131 Frederick, Jeanne M.; Thulin, Craig; Bernstein, Paul S.Identification and characterization of a Pi isoform of glutathione S-transferase (GSTP1) as a zeaxanthin-binding protein in the macula of the human eyeUptake, metabolism, and stabilization of xanthophyll carotenoids in the retina are thought to be mediated by specific xanthophyll-binding proteins (XBPs). A membrane-associated XBP was purified from human macula using ion-exchange chromatography followed by gel-exclusion chromatography. Two-dimensio...Circular Dichroism; Isoelectric Focusing; Macula Lutea; Xanthophylls2004
132 Baehr, Wolfgang; Prestwich, Glenn D.Photoreceptor cGMP phosphodiesterase delta subunit (PDEdelta) functions as a prenyl-binding proteinBovine PDEdelta was originally copurified with rod cGMP phosphodiesterase (PDE) and shown to interact with prenylated, carboxymethylated C-terminal Cys residues. Other studies showed that PDEdelta can interact with several small GTPases including Rab13, Ras, Rap, and Rho6, all of which are prenylate...Fluorescence Resonance Energy Transfer; GTP Phosphohydrolases; Immunohistochemistry2004
133 Zhang, KangExpression of wild type and mutant ELOVL4 in cell culture: subcellular localization and cell viabilityPURPOSE: ELOVL4 is a member of the fatty acid elongase (ELO) family of genes. Mutations of this gene are responsible for autosomal dominant Stargardt-like macular degeneration. However, the specific role of ELOVL4 in photoreceptor cells and the mechanism by which mutations in ELOVL4 causes macular d...Endoplasmic Reticulum; Green Fluorescent Proteins; Microscopy, Fluorescence2004
134 Zhang, Kang; Yang, Zhenglin; Jiang, LiMutations in LRP5 or FZD4 underlie the common familial exudative vitreoretinopathy locus on chromosome 11qFamilial exudative vitreoretinopathy (FEVR) is an inherited blinding disorder of the retinal vascular system. Autosomal dominant FEVR is genetically heterogeneous, but its principal locus, EVR1, is on chromosome 11q13-q23. The gene encoding the Wnt receptor frizzled-4 (FZD4) was recently reported ...Familial exudative vitreoretinopathy; FEVR; Inherited blinding disorders2004
135 Olson, Randall J.Clinical experience with 21-gauge manual microphacoemulsification using Sovereign WhiteStar Technology in eyes with dense cataractPURPOSE: To present a series of patients who had 21-gauge microphacoemulsification using Sovereign WhiteStar technology. SETTING: John A. Moran Eye Center, University of Utah School of Medicine, Salt Lake City, Utah, USA. METHODS: Eighteen consecutive patients with cataracts judged to be 3+ or 4+ on...Cataract; Microsurgery; Phacoemulsification; Postoperative Complications; Visual Acuity2004-01
136 Olson, Randall J.Legacy AdvanTec and Sovereign WhiteStar: a wound temperature studyPURPOSE: To assess the wound temperature of the Sovereign WhiteStar (S-WS) (AMO) and Legacy AdvanTec (L-ADV) (Alcon) phacoemulsification systems. SETTING: John A. Moran Eye Center, Health Sciences Center, University of Utah, Salt Lake City, Utah, USA. METHODS: Phacoemulsification using 20-gauge, 30-...Eye Burns; Wound Temperature; Phacoemulsification Systems2004-05
137 Digre, Kathleen B.; Blumenthal, Deborah T.; Cessna, Melissa H.; Gopez, Evelyn V.; Shen, YueCarcinomatous meningitis as the presenting manifestation of gallbladder carcinoma: case report and review of the literatureThe primary tumors that typically cause carcinomatous meningitis include lung cancer, breast cancer, leukemia, lymphoma and melanoma. A variety of neurological signs and symptoms can be seen depending on the extent and location of the meningeal metastasis. Once the diagnosis of carcinomatous meningi...Carcinomatous Meningitis; Gallbladder Carcinoma; Leptomeningeal Metastasis; Meningeal Carcinomatosis; Cranial Neuropathy2004-10
138 Olson, Randall J.Effect of lens edge design versus anterior capsule overlap on posterior capsule opacificationPURPOSE: To determine whether lens edge design or anterior capsule overlap on the intraocular lens (IOL) has greater effect on posterior capsule opacification (PCO). DESIGN: Retrospective cohort clinical study. METHODS: Retrospective. SETTING: Academic clinical practice. PATIENT POPULATION: The pati...Posterior Capsular Opacification' Intraocular Lens; Anterior Capsular Opacification; Acrylic Lenses; Silicone Lenses2004-10
139 Baehr, WolfgangNovel GCAP1 missense mutation (L151F) in a large family with autosomal dominant cone-rod dystrophy (adCORD)PURPOSE: To elucidate the phenotypic and biochemical characteristics of a novel mutation associated with autosomal dominant cone-rod dystrophy (adCORD). METHODS: Twenty-three family members of a CORD pedigree underwent clinical examinations, including visual acuity tests, standardized full-field ERG...Polymorphism, Single-Stranded Conformational; Guanylate Cyclase-Activating Proteins; DNA Mutational Analysis2005
140 Baehr, WolfgangRole of photoreceptor-specific retinol dehyrogenase (prRDH) in the retinoid cycle in vivoThe retinoid cycle is a recycling system that replenishes the 11-cis-retinal chromophore of rhodopsin and cone pigments. Photoreceptor-specific retinol dehydrogenase (prRDH) catalyzes reduction of all-trans-retinal to all-trans-retinol and is thought to be a key enzyme in the retinoid cycle. We d...Rhodopsin, Photoreceptors; prRDH (RDH8); All-trans-RDH; A2E; Retinoid cycle; Dark adaptation2005
141 Zhang, KangLoss 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
142 Bernstein, Paul S.; Zabriskie, Norman A.; Zhang, KangCFH Y402H Confers similar risk of soft Drusen and both forms of advanced AMDBACKGROUND: Age-related macular degeneration (AMD) is the most common cause of irreversible visual impairment in the developed world. The two forms of advanced AMD, geographic atrophy and neovascular AMD, represent different pathological processes in the macula that lead to loss of central vision. S...2005
143 Baehr, WolfgangEvaluation of the 17 kda prenyl binding protein as a regulatory protein for phototransduction in retinal photoreceptorsThe mammalian rod photoreceptor phosphodiesterase (PDE6) holoenzyme is isolated in both a membrane-associated and a soluble form. Membrane binding is a consequence of prenylation of PDE6 catalytic subunits, whereas soluble PDE6 is purified with a 17-kDa prenyl-binding protein (PDEdelta) tightly boun...Amino Acid Sequence; Immunohistochemistry; Phosphoric Diester Hydrolases2005
144 Normann, Richard A.Statistical encoding model for a primary motor cortical brain-computer interfaceA number of studies of the motor system suggest that the majority of primary motor cortical neurons represent simple movement-related kinematic and dynamic quantities in their time-varying activity patterns. An example of such an encoding relationship is the cosine tuning of firing rate with respect...Discrete Distribution; LN Model; Neural Decoding; Neuroprosthetics; Sequential Monte-Carlo Filter2005
145 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
146 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
147 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
148 Baehr, WolfgangMetabolism and transactivation activity of 13,14-dihydroretinoic acidThe metabolism of vitamin A is a highly regulated process that generates essential mediators involved in the development, cellular differentiation, immunity, and vision of vertebrates. Retinol saturase converts all-trans-retinol to all-trans-13,14-dihydroretinol (Moise, A. R., Kuksa, V., Imanishi, Y...Chromatography, High Pressure Liquid; Electrophoresis, Polyacrylamide Gel; Retinoid X Receptors2005
149 Baehr, Wolfgang; Katz, Bradley J.; Creel, Donnell J.; Zhang, KangAutosomal dominant cone dystrophy caused by a novel mutation in the GCAP1 gene (GUCA1A)PURPOSE: To describe the clinical features and genetic analysis of a family with an autosomal dominant cone dystrophy (adCD). METHODS: Selected members of a family with an autosomal dominant cone dystrophy underwent ophthalmic evaluation. Blood samples were obtained, genomic DNA was isolated, and ge...Mutation, Missense; DNA Mutational Analysis; Electroretinography2005
150 Olson, Randall J.Cohort study of 27 cases of endophthalmitis at a single institutionPURPOSE: To identify potential risk factors associated with post-cataract surgery bacterial endophthalmitis. SETTING: The John A. Moran Eye Center, Salt Lake City, Utah, USA. METHODS: This retrospective cohort study consisted of patients who had surgery for cataract(s) at this eye hospital. A 10% sa...Cataract Extraction; Endophthalmitis; Wound Healing; Risk Factors2005-04
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