keyword
MENU ▼
Read by QxMD icon Read
search

Fabry

keyword
https://www.readbyqxmd.com/read/29452394/treatment-of-hypertrophic-cardiomyopathy-caused-by-cardiospecific-variants-of-fabry-disease-with-chaperone-therapy
#1
Jonas Müntze, Tim Salinger, Daniel Gensler, Christoph Wanner, Peter Nordbeck
No abstract text is available yet for this article.
February 14, 2018: European Heart Journal
https://www.readbyqxmd.com/read/29447072/construction-and-structure-studies-of-dna-bipyridine-complexes-as-versatile-scaffolds-for-site-specific-incorporation-of-metal-ions-into-dna
#2
Rui Wang, Srivathsan V Ranganathan, Phensinee Haruehanroengra, Song Mao, Matteo Scalabrin, Daniele Fabris, Alan Chen, Hehua Liu, Abdalla E A Hassan, Jianhua Gan, Jia Sheng
The facile construction of metal-DNA complexes by using 'Click' reactions is reported here. A series of 2'-propargyl-modified DNA oligonucleotides were initially synthesized as structure scaffolds and were then modified through 'Click' reaction to incorporate a bipyridine ligand equipped with an azido group. These metal chelating ligands can be placed in the DNA context in site-specific fashion to provide versatile templates for binding various metal ions, which are exchangeable by using a simple EDTA washing-and-filtration step...
February 15, 2018: Journal of Biomolecular Structure & Dynamics
https://www.readbyqxmd.com/read/29446918/three-and-two-photon-nir-to-vis-yb-er-upconversion-from-ald-mld-fabricated-molecular-hybrid-thin-films
#3
Zivile Giedraityte, Minnea Tuomisto, Mika Lastusaari, Maarit Karppinen
We report blue, green and red upconversion emissions with strongly angular dependent intensities for new type of hybrid (Y,Yb,Er)-pyrazine thin films realized using the atomic/molecular layer deposition (ALD/MLD) thin-film fabri-cation technology. The luminescence emissions in our amorphous (Y,Yb,Er)-pyrazine thin films of a controllable na-nothickness originate from three- and two-photon NIR-to-vis excitation processes. In addition to shielding the lan-thanide ions from nonradiative de-excitation, the network of interconnected organic molecules serves as an excellent matrix for the Yb3+-to-Er3+ excitation energy transfer...
February 15, 2018: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/29445196/on-the-origins-of-transport-inefficiencies-in-mesoscopic-networks
#4
Sébastien Toussaint, Frederico Martins, Sébastien Faniel, Marco G Pala, Ludovic Desplanque, Xavier Wallart, Hermann Sellier, Serge Huant, Vincent Bayot, Benoit Hackens
A counter-intuitive behavior analogous to the Braess paradox is encountered in a two-terminal mesoscopic network patterned in a two-dimensional electron system (2DES). Decreasing locally the electron density of one channel of the network paradoxically leads to an increased network electrical conductance. Our low temperature scanning gate microscopy experiments reveal different occurrences of such puzzling conductance variations, thanks to tip-induced localized modifications of electron flow throughout the network's channels in the ballistic and coherent regime of transport...
February 14, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29444977/microenvironmental-regulation-of-the-il-23r-il-23-axis-overrides-chronic-lymphocytic-leukemia-indolence
#5
Giovanna Cutrona, Claudio Tripodo, Serena Matis, Anna Grazia Recchia, Carlotta Massucco, Marina Fabbi, Monica Colombo, Laura Emionite, Sabina Sangaletti, Alessandro Gulino, Daniele Reverberi, Rosanna Massara, Simona Boccardo, Daniela de Totero, Sandra Salvi, Michele Cilli, Mariavaleria Pellicanò, Martina Manzoni, Sonia Fabris, Irma Airoldi, Francesca Valdora, Silvano Ferrini, Massimo Gentile, Ernesto Vigna, Sabrina Bossio, Laura De Stefano, Angela Palummo, Giovanni Iaquinta, Martina Cardillo, Simonetta Zupo, Giannamaria Cerruti, Adalberto Ibatici, Antonino Neri, Franco Fais, Manlio Ferrarini, Fortunato Morabito
Although the progression of chronic lymphocytic leukemia (CLL) requires the cooperation of the microenvironment, the exact cellular and molecular mechanisms involved are still unclear. We investigated the interleukin (IL)-23 receptor (IL-23R)/IL-23 axis and found that circulating cells from early-stage CLL patients with shorter time-to-treatment, but not of those with a more benign course, expressed a defective form of the IL-23R complex lacking the IL-12Rβ1 chain. However, cells from both patient groups expressed the complete IL-23R complex in tissue infiltrates and could be induced to express the IL-12Rβ1 chain when cocultured with activated T cells or CD40L + cells...
February 14, 2018: Science Translational Medicine
https://www.readbyqxmd.com/read/29437868/agalsidase-alfa-versus-agalsidase-beta-for-the-treatment-of-fabry-disease-an-international-cohort-study
#6
Maarten Arends, Marieke Biegstraaten, Christoph Wanner, Sandra Sirrs, Atul Mehta, Perry M Elliott, Daniel Oder, Oliver T Watkinson, Daniel G Bichet, Aneal Khan, Mark Iwanochko, Frédéric M Vaz, André B P van Kuilenburg, Michael L West, Derralynn A Hughes, Carla E M Hollak
BACKGROUND: Two recombinant enzymes (agalsidase alfa 0.2 mg/kg/every other week and agalsidase beta 1.0 mg/kg/every other week) have been registered for the treatment of Fabry disease (FD), at equal high costs. An independent international initiative compared clinical and biochemical outcomes of the two enzymes. METHODS: In this multicentre retrospective cohort study, clinical event rate, left ventricular mass index (LVMI), estimated glomerular filtration rate (eGFR), antibody formation and globotriaosylsphingosine (lysoGb3) levels were compared between patients with FD treated with agalsidase alfa and beta at their registered dose after correction for phenotype and sex...
February 7, 2018: Journal of Medical Genetics
https://www.readbyqxmd.com/read/29436546/detailed-correlations-between-sers-enhancement-and-plasmon-resonances-in-subwavelength-closely-spaced-au-nanorod-arrays
#7
Yu Huang, Xian Zhang, Emilie Ringe, Lingwei Ma, Xiang Zhai, Lingling Wang, Zhengjun Zhang
Depending on the experimental conditions and plasmonic systems, the correlations between near-field surface enhanced Raman scattering (SERS) behaviors and far-field localized surface plasmon resonance (LSPR) responses have sometimes been accepted directly or argued or explored. In this work, we focus on the attractive subwavelength closely spaced metallic nanorod arrays and investigate in detail the complex relationship between their SERS behaviors and plasmon resonances. This is achieved utilizing a combination of array fabrication, conventional LSPR spectra, SERS measurements, electron microscopy and numerical modeling...
February 13, 2018: Nanoscale
https://www.readbyqxmd.com/read/29427798/evidence-for-multiple-sylvatic-transmission-cycles-during-the-2016-2017-yellow-fever-virus-outbreak-brazil
#8
Andres Moreira-Soto, Maria Celeste Torres, Marcos Cesar Lima de Mendonça, Maria Angelica Mares-Guia, Cintia Damasceno Dos Santos Rodrigues, Allison Fabri, Carolina Cardoso Dos Santos, Eliane Saraiva Machado Araújo, Carlo Fischer, Rita Maria Ribeiro Nogueira, Christian Drosten, Patricia Carvalho Sequeira, Jan Felix Drexler, Ana Maria Bispo de Filippis
OBJECTIVE: Since December 2016, Brazil has experienced an unusually large outbreak of yellow fever (YF). Whether urban transmission may contribute to the extent of the outbreak is unclear. The objective of this study was to characterize yellow fever virus (YFV) genomes and to identify spatial patterns to determine the distribution and origin of YF cases in Minas Gerais, Espírito Santo and Rio de Janeiro, the most affected Brazilian states during the current YFV outbreak. METHODS: We characterized near-complete YFV genomes from 14 human cases and two non-human primates (NHP), sampled from February to April 2017, retrieved epidemiological data of cases and used a geographic information system to investigate geospatial spread of YFV...
February 7, 2018: Clinical Microbiology and Infection
https://www.readbyqxmd.com/read/29424637/hysterothylacium-fabri-nematoda-raphidascarididae-in-mullus-surmuletus-perciformes-mullidae-and-uranoscopus-scaber-perciformes-uranoscopidae-from-the-mediterranean
#9
Perla Tedesco, Andrea Gustinelli, Monica Caffara, Pierpaolo Patarnello, Antonio Terlizzi, Maria L Fioravanti
Raphidascarididae are among the most abundant and widespread parasitic nematodes in the marine environment. Knowledge about the life-cycle of most raphidascaridid species is still poorly known and information about their distribution and host range is lacking in many geographical areas, as well as the taxonomy of several species. A study of larval and adult stages of Hysterothylacium fabri (Rudolphi, 1819) Deardorff & Overstreet, 1980 (Nematoda: Raphidascarididae) infecting the striped goatfish Mullus surmuletus Linnaeus, 1758 (Mullidae) and the Mediterranean stargazer Uranoscopus scaber Linnaeus, 1759 (Uranoscopidae) from Ionian Sea (central Mediterranean) has been carried out by combining light and scanning electron microscopy observations and molecular analyses through PCR-RFLP and sequencing of the ITS rDNA gene...
February 9, 2018: Journal of Parasitology
https://www.readbyqxmd.com/read/29422631/electrically-tunable-perfect-light-absorbers-as-color-filters-and-modulators
#10
Seyed Sadreddin Mirshafieyan, Don A Gregory
Methods for spectrally controlling light absorption in optoelectronic devices have attracted considerable attention in recent years. It is now well known that a Fabry-Perot nanocavity comprising thin semiconductor and metal films can be used to absorb light at selected wavelengths. The absorption wavelength is controlled by tailoring the thickness of the nanocavity and also by nanostructure patterning. However, the realization of dynamically tuning the absorption wavelength without changing the structural geometry remains a great challenge in optoelectronic device development...
February 8, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29421273/characterization-of-drug-neutralizing-antibodies-in-patients-with-fabry-disease-during-infusion
#11
Malte Lenders, Boris Schmitz, Stefan Martin Brand, Dirk Foell, Eva Brand
In patients with Fabry disease, neutralizing anti-drug antibodies were identified as IgG4 isotypes. The determination of antibody titers during infusions indicated that high dosages of enzyme overcome anti-drug antibody titers, necessitating individually tailored dose adjustment.
February 5, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/29421001/thrombus-aspiration-and-prehospital-ticagrelor-administration-in-st-elevation-myocardial-infarction-findings-from-the-atlantic-trial
#12
Sinem Kilic, Enrico Fabris, Arnoud W J Van't Hof, Christian W Hamm, Frédéric Lapostolle, Jens Flensted Lassen, Anne Tsatsaris, Abdourahmane Diallo, Eric Vicaut, Gilles Montalescot
BACKGROUND: The potential interactions between prehospital (pre-H) ticagrelor administration and thrombus aspiration (TA) in patients with ST-elevation myocardial infarction (STEMI) treated with primary percutaneous coronary intervention (PCI) have never been studied. Therefore, we evaluated the potential benefit of TA and pre-H ticagrelor treatment in patients enrolled in the ATLANTIC trial (NCT01347580). METHODS: This analysis included 1,630 patients who underwent primary PCI...
February 2018: American Heart Journal
https://www.readbyqxmd.com/read/29419745/optical-fiber-sensors-based-on-fiber-ring-laser-demodulation-technology
#13
REVIEW
Wen-Ge Xie, Ya-Nan Zhang, Peng-Zhao Wang, Jian-Zhang Wang
A review for optical fiber sensors based on fiber ring laser (FRL) demodulation technology is presented. The review focuses on the principles, main structures, and the sensing performances of different kinds of optical fiber sensors based on FRLs. First of all, the theory background of the sensors has been discussed. Secondly, four different types of sensors are described and compared, which includes Mach-Zehnder interferometer (MZI) typed sensors, Fabry-Perot interferometer (FPI) typed sensors, Sagnac typed sensors, and fiber Bragg grating (FBG) typed sensors...
February 8, 2018: Sensors
https://www.readbyqxmd.com/read/29415625/a-predominant-cardiac-phenotype-of-anderson-fabry-disease-in-presence-of-a-mybpc3-gene-mutation-and-a-lama4-gene-mutation
#14
Dorien Laenens, Pieter Koopman, Thijs Cools
No abstract text is available yet for this article.
February 7, 2018: Acta Cardiologica
https://www.readbyqxmd.com/read/29407357/measuring-racing-thoughts-in-healthy-individuals-the-racing-and-crowded-thoughts-questionnaire-rctq
#15
Luisa Weiner, Sébastien Weibel, Wagner de Sousa Gurgel, Ineke Keizer, Marianne Gex-Fabry, Anne Giersch, Gilles Bertschy
Racing thoughts refer to an acceleration and overproduction of thoughts, which have been associated with manic and mixed episodes. Phenomenology distinguishes 'crowded' from 'racing' thoughts, associated with mixed depression and mania, respectively. Recent data suggest racing thoughts might also be present in healthy individuals with sub-affective traits and symptoms. We investigated this assumption, with a 34-item self-rating scale, the Racing and Crowded Thoughts Questionnaire (RCTQ), and evaluated its reliability, factor structure, and concurrent validity...
January 26, 2018: Comprehensive Psychiatry
https://www.readbyqxmd.com/read/29402025/harmonically-mode-locked-yb-calgo-laser-pumped-by-a-single-mode-1-2-w-laser-diode
#16
Sha Wang, Yan-Biao Wang, Guo-Ying Feng, Shou-Huan Zhou
We present a SESAM mode locked Yb:CALGO laser with a harmonic repetition rate to the 300th order pumped by a single-mode fiber coupled laser diode. By fine tuning the internal angle between the laser beam and the normal axis through the gain medium, at pump power of 1.2 W, an average output power of 132 mW is achieved with a pulse duration of 777.6 fs and a repetition rate of 22.4 GHz. The amplification effect over several tens of roundtrips induced Fabry-Perot filtering of the anti-reflection (AR) coated gain medium is analyzed...
January 22, 2018: Optics Express
https://www.readbyqxmd.com/read/29401924/mechano-optical-effects-in-multiwall-carbon-nanotubes-ethanol-based-nanofluids
#17
J A García-Merino, C Mercado-Zúñiga, C R Torres-Sanmiguel, C Torres-Torres
A highly sensitive technique for analyzing surface tension and dynamic viscosity of nanofluids was reported. Multiwall carbon nanotubes suspended in ethanol were evaluated. The assistance of a Fabry-Perot interferometer integrated by a small sample volume fluid allowed us to explore the stability and mechanical properties exhibited by the nanostructures. The surface tension and dynamic viscosity of the colloid was examined by using interferometric optical signals reflected from a remnant drop pending at the end of an optical fiber...
January 22, 2018: Optics Express
https://www.readbyqxmd.com/read/29401907/silicon-optical-filter-with-transmission-peaks-in-wide-stopband-obtained-by-anti-symmetric-photonic-crystal-with-defect-in-multimode-waveguides
#18
Manuel Mendez-Astudillo, Hideaki Okayama, Hirochika Nakajima
We experimentally demonstrate and characterize a wide optical stopband filter with transmission peaks constituted by two parallel rows of an anti-symmetric one-dimensional (1D) photonic crystal with defect in a multimode waveguide. The working principle is based on mode coupling for the wavelengths that meet the phase matching condition as the fundamental mode is coupled to a higher-order mode and then filtered out with linear tapers as they are reflected back. The defect in the photonic crystal works as a Fabry-Perot like cavity that allows for localized states in the stopband to quantify the effects of the design parameters and we show a Fabry-Perot filter that extends through the 200 nm stopband...
January 22, 2018: Optics Express
https://www.readbyqxmd.com/read/29401898/mechanism-of-propagating-graphene-plasmons-excitation-for-tunable-infrared-photonic-devices
#19
Linlong Tang, Wei Wei, Xingzhan Wei, Jinpeng Nong, Chunlei Du, Haofei Shi
The mechanism of propagating graphene plasmons excitation using a nano-grating and a Fabry-Pérot cavity as the optical coupling components is studied. It is demonstrated that the system could be well described within the temporal coupled mode theory using two phenomenological parameters, namely, the intrinsic loss rate and the coupling rate of a graphene plasmonic mode, and their analytical expressions are derived. It is found that the intrinsic loss rate is solely determined by the electron relaxation time of graphene, while independent of the field distributions of the modes...
February 5, 2018: Optics Express
https://www.readbyqxmd.com/read/29401878/enhanced-asymmetric-transmissions-attributed-to-the-cavity-coupling-hybrid-resonance-in-a-continuous-omega-shaped-metamaterial
#20
Ying-Hua Wang, Ren-Chao Jin, Jie Li, Jia-Qi Li, Zheng-Gao Dong
In this paper, the infinite-length metallic bar is folded to a continuous omega-shaped resonator and then arranged as a bi-layer metamaterial, which presents a hybrid resonance and a Fabry-Perot-like cavity mode. The asymmetric transmission (AT) for linearly polarized light is powerfully enhanced at a near-infrared regime by strongly coupling the hybrid resonance to the cavity, with the maximum value of the high-efficiency AT effect reaching 0.8 at around 1364 nm. At this near-infrared band, such a high-efficiency AT effect has never been realized previously by a bi-layer metamaterial...
February 5, 2018: Optics Express
keyword
keyword
2170
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"