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https://www.readbyqxmd.com/read/29131863/long-term-retinal-cone-rescue-using-a-capsid-mutant-aav8-vector-in-a-mouse-model-of-cnga3-achromatopsia
#1
Xufeng Dai, Ying He, Hua Zhang, Yangyang Zhang, Yan Liu, Muran Wang, Hao Chen, Ji-Jing Pang
Adeno-associated virus (AAV) vectors are important gene delivery tools for the treatment of many recessively inherited retinal diseases. For example, a wild-type (WT) AAV5 vector can deliver a full-length Cnga3 (cyclic nucleotide-gated channel alpha-3) cDNA to target cells of the cone photoreceptor function loss 5 (cpfl5) mouse, a spontaneous animal model of achromatopsia with a Cnga3 mutation. Gene therapy restores cone-mediated function and blocks cone degeneration in the mice. However, since transgene expression delivered by an AAV vector shows relatively short-term effectiveness, this cannot be regarded as a very successful therapy...
2017: PloS One
https://www.readbyqxmd.com/read/29130140/transforaminal-intrathecal-delivery-of-nusinersen-using-cone-beam-computed-tomography-for-children-with-spinal-muscular-atrophy-and-extensive-surgical-instrumentation-early-results-of-technical-success-and-safety
#2
John J Weaver, Niranjana Natarajan, Dennis W W Shaw, Susan D Apkon, Kevin S H Koo, Giri M Shivaram, Eric J Monroe
BACKGROUND: Nusinersen, the only treatment approved by the United States Food and Drug Administration for spinal muscular atrophy (SMA), is delivered intrathecally. Many children with SMA have extensive spinal instrumentation and deformities, often precluding the use of standard approaches for gaining intrathecal access. Furthermore the anatomical distortion that often occurs with rotoscoliosis can complicate the use of fluoroscopic guidance. Compared to fluoroscopy, CT affords superior guidance for complex needle placements...
November 13, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/29129732/chromatic-summation-and-receptive-field-properties-of-blue-on-and-blue-off-cells-in-marmoset-lateral-geniculate-nucleus
#3
C D Eiber, A N J Pietersen, N Zeater, S G Solomon, P R Martin
The "blue-on" and "blue-off" receptive fields in retina and dorsal lateral geniculate nucleus (LGN) of diurnal primates combine signals from short-wavelength sensitive (S) cone photoreceptors with signals from medium/long wavelength sensitive (ML) photoreceptors. Three questions about this combination remain unresolved. Firstly, is the combination of S and ML signals in these cells linear or non-linear? Secondly, how does the timing of S and ML inputs to these cells influence their responses? Thirdly, is there spatial antagonism within S and ML subunits of the receptive field of these cells? We measured contrast sensitivity and spatial frequency tuning for four types of drifting sine gratings: S cone isolating, ML cone isolating, achromatic (S + ML), and counterphase chromatic (S - ML), in extracellular recordings from LGN of marmoset monkeys...
November 9, 2017: Vision Research
https://www.readbyqxmd.com/read/29124762/evolution-in-the-design-of-a-low-sheath-flow-interface-for-ce-ms-and-application-to-biological-samples
#4
Víctor González-Ruiz, Santiago Codesido, Serge Rudaz, Julie Schappler
Although several interfaces for CE-MS hyphenation are commercially available, the development of new versatile, simple and yet efficient and sensitive alternatives remains an important field of research. In a previous work, a simple low sheath-flow interface was developed from inexpensive parts. This interface features a design easy to build, maintain, and adapt to particular needs. The present work introduces an improved design of the previous interface. By reducing the diameter of the separation capillary and the emitter, a smaller Taylor cone is spontaneously formed, minimizing the zone dispersion while the analytes go through the interface and leading to less peak broadening associated to the ESI process...
November 10, 2017: Electrophoresis
https://www.readbyqxmd.com/read/29122423/efferent-modulation-of-pre-neural-and-neural-distortion-products
#5
S B Smith, K Ichiba, D S Velenovsky, B Cone
Distortion product otoacoustic emissions (DPOAEs) and distortion product frequency following responses (DPFFRs) are respectively pre-neural and neural measurements associated with cochlear nonlinearity. Because cochlear nonlinearity is putatively linked to outer hair cell electromotility, DPOAEs and DPFFRs may provide complementary measurements of the human medial olivocochlear (MOC) reflex, which directly modulates outer hair cell function. In this study, we first quantified MOC reflex-induced DPOAE inhibition at spectral fine structure peaks in 22 young human adults with normal hearing...
October 26, 2017: Hearing Research
https://www.readbyqxmd.com/read/29122074/cone-beam-computed-tomography-evaluation-of-hand-nickel-titanium-k-files-and-rotary-system-in-primary-teeth
#6
Etta Radhika, E Rajendra Reddy, S Thabitha Rani, Labishetty Vinay Kumar, M Manjula, Tulluru Ajay Mohan
PURPOSE: The purpose of this study was to compare canal transportation, centering ability, and removed dentin thickness (RDT) using hand nickel-titanium (NiTi) K-flex files and rotary systems in primary molars and using cone beam computed tomography (CBCT). METHODS: This study was conducted on 20 primary maxillary and 20 primary mandibular molars, which were randomly divided into two groups to be instrumented with hand NiTi K-flex files and a rotary system. Pre- and post-instrumentation slices were taken at three predetermined levels using CBCT to evaluate all the parameters by using Digital Imaging Communications in Medicine (DICOM) software...
July 15, 2017: Pediatric Dentistry
https://www.readbyqxmd.com/read/29120066/a-novel-nonsense-variant-in-reep6-is-involved-in-a-sporadic-rod-cone-dystrophy-case
#7
C Méjécase, S Mohand-Saïd, S El Shamieh, A Antonio, C Condroyer, S Blanchard, M Letexier, J-P Saraiva, J-A Sahel, I Audo, C Zeitz
Rod-cone dystrophy (RCD), also called retinitis pigmentosa, is the most common form of progressive inherited retinal disorders secondary to photoreceptor degeneration. It is a genetically heterogeneous disease characterized by night blindness, followed by visual field constriction and, in most severe cases, total blindness. The aim of our study was to identify the underlying gene defect leading to severe RCD in a 60-year-old woman. The patient's DNA was investigated by targeted next generation sequencing followed by whole exome sequencing...
November 9, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/29108580/development-of-flow-systems-incorporating-membraneless-vaporization-units-and-flow-through-contactless-conductivity-detector-for-determination-of-dissolved-ammonium-and-sulfide-in-canal-water
#8
Waleed Alahmad, Thanakorn Pluangklang, Thitirat Mantim, Victor Cerdà, Prapin Wilairat, Nuanlaor Ratanawimarnwong, Duangjai Nacapricha
Use of membraneless vaporization (MBL-VP) unit with two cone-shaped reservoirs is presented for on-line separation and detection of non-volatile species. A flow system comprising two sets of MBL-VP units with a single in-house capacitively coupled contactless conductivity detector (C4D) was developed for dual determination of ammonium and sulfide ions. Using the continuous-flow section, two zones (280μL) of a sample, either mixed with sodium hydroxide (for ammonium) or hydrochloric acid (for sulfide), are separately delivered into the donor reservoir of the MBL-VP units...
January 15, 2018: Talanta
https://www.readbyqxmd.com/read/29106967/asymmetric-high-contrast-masking-in-s-cone-increment-and-decrement-pathways
#9
Scott H Gabree, Timothy G Shepard, Rhea T Eskew
Physiological, anatomical, and psychophysical evidence points to important differences between visual processing of short-wave cone increments and decrement (S+ and S-) stimuli. The present study uses the pedestal discrimination paradigm to investigate potential differences, using S+ and S- tests presented on (L)ong-wave, (M)edium-wave, S, L+M, L-M, and achromatic pedestals, of both contrast polarities. Results show that high contrast 'purplish' (S+ or -(L+M)) pedestals produce substantially more masking of both S+ and S- tests than 'yellowish' (S- or +(L+M)) pedestals do...
October 26, 2017: Vision Research
https://www.readbyqxmd.com/read/29100788/deep-inspiration-breath-hold-intensity-modulated-radiation-therapy-to-the-mediastinum-for-lymphoma-patients-setup-uncertainties-and-margins
#10
Michalis Aristophanous, Pai-Chun M Chi, Jeremy Kao, Ryan Williamson, Sam Tung, Therese Andraos, Sarah A Milgrom, Chelsea C Pinnix, Bouthaina S Dabaja
PURPOSE: Patient setup for treating large target volumes can be challenging. In the present study, we measured the local uncertainties in the treatment of mediastinal lymphoma and investigated the need for region-specific planning target volume (PTV) margins. METHODS AND MATERIALS: The data from 30 patients who had undergone radiation therapy for mediastinal lymphoma were retrospectively analyzed. A computed tomography (CT)-on-rails (CTOR) system in the treatment room was used for daily image guidance...
September 25, 2017: International Journal of Radiation Oncology, Biology, Physics
https://www.readbyqxmd.com/read/29099202/importance-of-%C3%AF-bonding-electrons-for-the-accurate-description-of-electron-correlation-in-graphene
#11
Huihuo Zheng, Yu Gan, Peter Abbamonte, Lucas K Wagner
Electron correlation in graphene is unique because of the interplay between the Dirac cone dispersion of π electrons and long-range Coulomb interaction. Because of the zero density of states at Fermi level, the random phase approximation predicts no metallic screening at long distance and low energy, so one might expect that graphene should be a poorly screened system. However, empirically graphene is a weakly interacting semimetal, which leads to the question of how electron correlations take place in graphene at different length scales...
October 20, 2017: Physical Review Letters
https://www.readbyqxmd.com/read/29079576/ablation-of-the-riboflavin-binding-protein-retbindin-reduces-flavin-levels-and-leads-to-progressive-and-dose-dependent-degeneration-of-rods-and-cones
#12
Ryan A Kelley, Muayyad R Al-Ubaidi, Tirthankar Sinha, Ayse M Genc, Mustafa S Makia, Larissa Ikelle, Muna I Naash
The interface between the neural retina and the retinal pigment epithelium (RPE) is critical for several processes, including visual pigment regeneration and retinal attachment to the RPE. One of its most important functions is the exchange of metabolites between the photoreceptors and RPE because photoreceptor cells have very high energy demands, largely satisfied by oxidative metabolism. The riboflavin (RF) cofactors, flavin adenine dinucleotide (FAD) and flavin mononucleotide (FMN) are two key cofactors involved in oxidative metabolism...
October 27, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/29078372/dephosphorylation-by-protein-phosphatase-2a-regulates-visual-pigment-regeneration-and-the-dark-adaptation-of-mammalian-photoreceptors
#13
Alexander V Kolesnikov, Tivadar Orban, Hui Jin, Celine Brooks, Lukas Hofmann, Zhiqian Dong, Maxim Sokolov, Krzysztof Palczewski, Vladimir J Kefalov
Resetting of G-protein-coupled receptors (GPCRs) from their active state back to their biologically inert ground state is an integral part of GPCR signaling. This "on-off" GPCR cycle is regulated by reversible phosphorylation. Retinal rod and cone photoreceptors arguably represent the best-understood example of such GPCR signaling. Their visual pigments (opsins) are activated by light, transduce the signal, and are then inactivated by a GPCR kinase and arrestin. Although pigment inactivation by phosphorylation is well understood, the enzyme(s) responsible for pigment dephosphorylation and the functional significance of this reaction remain unknown...
October 23, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29076751/analyses-of-aerodynamic-characteristics-of-the-oropharynx-applying-cbct-obstructive-sleep-apnea-patients-versus-control-subjects
#14
Hui Chen, Yingguang Li, Johan Hc Reiber, Jan de Lange, Shengxian Tu, Paul van der Stelt, Frank Lobbezoo, Ghizlane Aarab
OBJECTIVES: To determine the most relevant aerodynamic characteristic of the oropharynx related to the collapse of the upper airway in obstructive sleep apnea (OSA) patients; and to determine the correlation between the most relevant aerodynamic characteristic(s) of the oropharynx and anatomical characteristics of the oropharynx in OSA patients. METHODS: 31 mild to moderate OSA patients (mean±SD age = 43.5±9.7 yrs) and 13 control subjects (mean±SD age = 48.5±16...
October 27, 2017: Dento Maxillo Facial Radiology
https://www.readbyqxmd.com/read/29076746/predicting-poor-outcome-from-simple-ankle-injuries-a-prospective-cohort-study
#15
Stuart A Bullock, Georgina M Allen, Marion S Watson, David J Wilson
BACKGROUND: Sprained ankles are common and when there are no signs or evidence of a fracture, conventional management is conservative. At present, there are no clinical markers to identify those that may develop persisting instability and disability that would require rehabilitation or surgery. OBJECTIVES: To investigate the nature and extent of soft tissue ankle injuries, and to consider whether the anatomical severity of injury can predict functional outcome. METHODS: Patients attending a local Accident and Emergency Department in Oxford with an acute ankle injury with no clinical requirement for radiographs, or where radiographs were normal, were invited into the study...
November 9, 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/29073362/effectiveness-of-contour-augmentation-with-guided-bone-regeneration-10-year-results
#16
V Chappuis, L Rahman, R Buser, S F M Janner, U C Belser, D Buser
In aesthetic sites, the integrity of the facial bone wall dimension in the anterior maxilla is jeopardized by physiologic and structural changes postextraction. An effective regenerative protocol is key to reestablish and maintain the hard and soft tissue dimensions over time. The present prospective case series study examined the effectiveness of early implant placement with simultaneous contour augmentation through guided bone regeneration with a 2-layer composite graft in postextraction single-tooth sites over an observation period of 10 y among 20 patients...
October 1, 2017: Journal of Dental Research
https://www.readbyqxmd.com/read/29072647/-comparative-biomechanical-study-of-surgically-assisted-rapid-palatal-expansion-with-tooth-borne-and-bone-borne-expanders
#17
A V Nikolaev, A R Andreishchev, S I Kutukova
The study objective was to compare the biomechanics of surgically assisted rapid palatal expansion (SARPE) with tooth-borne and bone-borne expanders. Materials include a sample of 54 cone-beam CT of 27 patients who underwent SARPE (bone-borne, n=21; tooth-borne, n=6) before treatment and at expander removal. Expansion efficacy was estimated by interapecal and intercoronal distances between maxillary canines, premolars and first molars as well as Coronal-Apical Index (CAI = coronal expansion (mm)/apical expansion (mm))...
2017: Stomatologii︠a︡
https://www.readbyqxmd.com/read/29072226/retrospective-morphometric-analysis-of-the-infraorbital-foramen-with-cone-beam-computed-tomography
#18
S Dagistan, Ö Miloǧlu, O Altun, E K Umar
OBJECTIVE: The aim of our study is to examine the morphometric characteristics of the infraorbital foramen (IOF) and its anatomic localization by using cone-beam computerized tomography (CBCT). MATERIALS AND METHODS: In our study, the anatomic characteristics of the IOF were identified by studying retrospectively the CBCT images of one hundred twenty-five (125) patients. These characteristics were assessed statistically in terms of age, gender, and right-left side...
September 2017: Nigerian Journal of Clinical Practice
https://www.readbyqxmd.com/read/29064779/anatomic-customization-of-root-analog-dental-implants-with-cone-beam-ct-and-cad-cam-fabrication-a-cadaver-based-pilot-evaluation
#19
Zachary Presson Evans, Walter G Renne, Thierry R Bacro, Anthony S Mennito, Mark E Ludlow, Michael K Lecholop
Existing root-analog dental implant systems have no standardized protocols relative to retentive design, surface manipulation, or prosthetic attachment design relative to the site's unique anatomy. Existing systems historically made these design choices arbitrarily. For this report, strategies were developed which deliberately reference the adjacent anatomy, implant and restorable path of draw, and bone density for the purposes of implant and retentive design. For proof-of-concept, dentate arches from human cadavers were scanned with cone-beam computed tomography and digitally modeled...
October 24, 2017: Journal of Oral Implantology
https://www.readbyqxmd.com/read/29062223/enhancing-the-efficacy-of-areds-antioxidants-in-light-induced-retinal-degeneration
#20
Paul Wong, M Markey, C M Rapp, R M Darrow, A Ziesel, D T Organisciak
PURPOSE: Light-induced photoreceptor cell degeneration and disease progression in age-related macular degeneration (AMD) involve oxidative stress and visual cell loss, which can be prevented, or slowed, by antioxidants. Our goal was to test the protective efficacy of a traditional Age-related Eye Disease Study antioxidant formulation (AREDS) and AREDS combined with non-traditional antioxidants in a preclinical animal model of photooxidative retinal damage. METHODS: Male Sprague-Dawley rats were reared in a low-intensity (20 lux) or high-intensity (200 lux) cyclic light environment for 6 weeks...
2017: Molecular Vision
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