keyword
MENU ▼
Read by QxMD icon Read
search

Fabrys

keyword
https://www.readbyqxmd.com/read/30106942/enhancement-of-the-lasing-efficiency-of-vitamin-b-2-in-a-highly-polar-organic-solvent-via-dna-lipid-complex
#1
Woohyun Jung, Byung Joo Kong, Seongjin Hong, Taeoh Kim, Kyunghwan Oh
We experimentally demonstrated a green liquid laser at the wavelength of 570 nm, utilizing the optical gain of vitamin B2 in a highly polar organic solvent, and proposed an efficient method to enhance its lasing efficiency by adding DNA-lipid complex (DNA-CTMA) in the solution. Optical properties of vitamin B2 in the hexafluoro-2-propanol solvents were investigated by adding various amounts of DNA-CTMA in terms of the UV-visible absorbance, the visible emission, and the fluorescence lifetime. A Fabry-Perot cavity was built to obtain the laser oscillation at 570 nm using a pulsed pump source at the wavelength of 450 nm, 5 ns pulse duration, and 10 Hz repetition rate...
August 15, 2018: Optics Letters
https://www.readbyqxmd.com/read/30103270/genomic-screening-of-fabry-disease-in-young-stroke-patients-the-taiwan-experience-and-a-review-of-the-literature
#2
Tsong-Hai Lee, Jen-Tsung Yang, Jiann-Der Lee, Ku-Chou Chang, Tsung-I Peng, Ting-Yu Chang, Kuo-Lun Huang, Chi-Hung Liu, Shan-Jin Ryu, Alessandro P Burlina
BACKGROUND: Fabry disease is a X-linked disease, and enzyme-based screening methods are not suitable for female patients. METHODS: Totally, 1,000 young stroke patients (18-55 y/o, 661 with ischemic stroke and 339 with hypertensive intracerebral hemorrhage) were recruited. Sequenom iPLEX assay was used to detect 26 Fabry related mutation genes. The frequency of Fabry disease in young stroke was reviewed and compared between Asian and non-Asian countries. RESULTS: Two male patients with ischemic stroke were found to have genetic mutation of IVS4+919G>A...
August 13, 2018: European Journal of Neurology: the Official Journal of the European Federation of Neurological Societies
https://www.readbyqxmd.com/read/30099469/systematic-dna-study-for-fabry-disease-in-the-end-stage-renal-disease-patients-from-a-southern-italy-area
#3
Carmela Zizzo, Alessandra Testa, Paolo Colomba, Maurizio Postorino, Giuseppe Natale, Alessandro Pini, Daniele Francofonte, Giuseppe Cammarata, Simone Scalia, Serafina Sciarrino, Carmine Zoccali, Giovanni Duro
BACKGROUND/AIMS: Fabry disease (FD) is a lysosomal storage disorder characterized by pervasive renal involvement. However, this disease is underdiagnosed in patient with chronic kidney disease (CKD), including those with end stage renal disease (ESRD), so their investigation represents an unexploited opportunity for early diagnosis of the disease and for its identification in relatives of affected patients. METHODS: We investigated Fabry disease in a clinical and biological database including ESRD patients of unknown cause in a geographical area with 2 million residents...
August 10, 2018: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/30096595/anisakid-and-raphidascaridid-nematodes-ascaridoidea-infection-in-the-important-marine-food-fish-lophius-litulon-jordan-lophiiformes-lophiidae
#4
Kuang Zhang, Zhen Xu, Hui-Xia Chen, Ning Guo, Liang Li
The yellow goosefish Lophius litulon is a commercially important marine fish species. However, our present knowledge of the occurrence of nematode parasites in L. litulon remains incomplete. A total of 42 L. litulon collected from the East China Sea were investigated for the presence of ascaridoid nematodes. Eight ascaridoid species with different developmental stages were identified using morphological and molecular methods, including Anisakis pegreffii (L3s), Raphidascaris lophii (L3s), Hysterothylacium fabri (L3s, L4s), H...
August 3, 2018: International Journal of Food Microbiology
https://www.readbyqxmd.com/read/30093709/the-new-york-pilot-newborn-screening-program-for-lysosomal-storage-diseases-report-of-the-first-65-000-infants
#5
Melissa P Wasserstein, Michele Caggana, Sean M Bailey, Robert J Desnick, Lisa Edelmann, Lissette Estrella, Ian Holzman, Nicole R Kelly, Ruth Kornreich, S Gabriel Kupchik, Monica Martin, Suhas M Nafday, Randi Wasserman, Amy Yang, Chunli Yu, Joseph J Orsini
PURPOSE: We conducted a consented pilot newborn screening (NBS) for Pompe, Gaucher, Niemann-Pick A/B, Fabry, and MPS 1 to assess the suitability of these lysosomal storage disorders (LSDs) for public health mandated screening. METHODS: At five participating high-birth rate, ethnically diverse New York City hospitals, recruiters discussed the study with postpartum parents and documented verbal consent. Screening on consented samples was performed using multiplexed tandem mass spectrometry...
August 10, 2018: Genetics in Medicine: Official Journal of the American College of Medical Genetics
https://www.readbyqxmd.com/read/30093456/effects-of-enzyme-replacement-therapy-and-antidrug-antibodies-in-patients-with-fabry-disease
#6
REVIEW
Malte Lenders, Eva Brand
No abstract text is available yet for this article.
August 9, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/30092286/glucocorticoids-promote-intrinsic-human-th17-differentiation
#7
Juliana de Castro Kroner, Kristin Knoke, David M Kofler, Julia Steiger, Mario Fabri
Glucocorticoids (GCs) trigger human Th17 differentiation, a process in which IL-2 regulation plays a mechanistic role. Th17 memory cells from healthy donors are similarly favored by GCs, but this is not observed in psoriatic patients.
August 6, 2018: Journal of Allergy and Clinical Immunology
https://www.readbyqxmd.com/read/30087087/reduced-incidence-of-cardiovascular-events-in-hyper-lp-a-patients-on-lipoprotein-apheresis-the-g-i-l-a-gruppo-interdisciplinare-aferesi-lipoproteica-pilot-study
#8
Federico Bigazzi, Francesco Sbrana, Daniele Berretti, Zenti Maria Grazia, Sabina Zambon, Antonia Fabris, Maurizio Fonda, Giovanni B Vigna, Giovanna D'Alessandri, Stefano Passalacqua, Beatrice Dal Pino, Mascia Pianelli, Roberta Luciani, Andrea Ripoli, Daniela Rafanelli, Enzo Manzato, Luigi Cattin, Tiziana Sampietro
BACKGROUND: Lipoprotein apheresis (LA) is the elective therapy for homozygous and other forms of Familial Hypercholesterolemia, Familial Combined Hypercholesterolemia, resistant/intolerant to lipid lowering drugs, and hyper-lipoproteinemia(a). Lipoprotein(a) [Lp(a)] has been classified as the most prevalent genetic risk factor for coronary artery disease and aortic valve stenosis. AIM: Our multicenter retrospective study has the aim to analyze the incidence of adverse cardiovascular events (ACVE) before and during the LA treatment, in subjects with elevated level of Lp(a) (>60 mg/dL) [hyper-Lp(a)] and chronic ischemic heart disease...
August 1, 2018: Transfusion and Apheresis Science
https://www.readbyqxmd.com/read/30085001/prominent-longitudinal-strain-reduction-of-left-ventricular-basal-segments-in-treatment-na%C3%A3-ve-anderson-fabry-disease-patients
#9
Roberta Esposito, Maurizio Galderisi, Ciro Santoro, Massimo Imbriaco, Eleonora Riccio, Angela Maria Pellegrino, Regina Sorrentino, Maria Lembo, Rodolfo Citro, Maria Angela Losi, Letizia Spinelli, Bruno Trimarco, Antonio Pisani
Aims: Little is known about regional longitudinal strain (LS) distribution in early stages of Anderson-Fabry disease (AFD) cardiomyopathy. We investigated regional left ventricular (LV) patterns of LS strain and base-to-apex behaviour of LS in treatment-naïve AFD patients. Methods and results: Twenty-three consecutive AFD patients at diagnosis and 23 healthy controls without cardiovascular risk factors and matched for age and sex to the patients, underwent a comprehensive evaluation of target organs...
August 6, 2018: European Heart Journal Cardiovascular Imaging
https://www.readbyqxmd.com/read/30083389/using-quantitative-systems-pharmacology-to-evaluate-the-drug-efficacy-of-cox-2-and-5-lox-inhibitors-in-therapeutic-situations
#10
Christoph Thiel, Ines Smit, Vanessa Baier, Henrik Cordes, Brigida Fabry, Lars Mathias Blank, Lars Kuepfer
A quantitative analysis of dose-response relationships is essential in preclinical and clinical drug development in order to optimize drug efficacy and safety, respectively. However, there is a lack of quantitative understanding about the dynamics of pharmacological drug-target interactions in biological systems. In this study, a quantitative systems pharmacology (QSP) approach is applied to quantify the drug efficacy of cyclooxygenase-2 (COX-2) and 5-lipoxygenase (5-LOX) inhibitors by coupling physiologically based pharmacokinetic models, at the whole-body level, with affected biological networks, at the cellular scale...
2018: NPJ Systems Biology and Applications
https://www.readbyqxmd.com/read/30082620/tunable-fabry-perot-interferometer-designed-for-far-infrared-wavelength-by-utilizing-electromagnetic-force
#11
Dong Geon Jung, Jun Yeop Lee, Jae Keon Kim, Daewoong Jung, Seong Ho Kong
A tunable Fabry-Perot interferometer (TFPI)-type wavelength filter designed for the long-wavelength infrared (LWIR) region is fabricated using micro electro mechanical systems (MEMS) technology and the novel polydimethylsiloxane (PDMS) micro patterning technique. The structure of the proposed infrared sensor consists of a Fabry-Perot interferometer (FPI)-based optical filter and infrared (IR) detector. An amorphous Si-based thermal IR detector is located under the FPI-based optical filter to detect the IR-rays filtered by the FPI...
August 6, 2018: Sensors
https://www.readbyqxmd.com/read/30080595/cell-wall-features-transferred-from-common-into-durum-wheat-to-improve-fusarium-head-blight-resistance
#12
Angelica Giancaspro, Vincenzo Lionetti, Stefania L Giove, Daniela Zito, Eleonora Fabri, Nathan Reem, Olga A Zabotina, Elisabetta De Angelis, Linda Monaci, Daniela Bellincampi, Agata Gadaleta
Durum wheat is naturally more susceptible to Fusarium graminerum infection in comparison to common wheat. The improvement of durum wheat resistance against F. graminearum is a challenge due to the lack of resistance sources in its gene pool. FHB-resistance factors were introduced in durum wheat by generating recombinant inbred lines (RILs), obtained by crossing the hexaploid resistant accession 02-5B-318 with the susceptible durum wheat cv. Saragolla. In this work we explored the possible contribution of cell wall (CW) in RILs with improved FHB resistance...
September 2018: Plant Science: An International Journal of Experimental Plant Biology
https://www.readbyqxmd.com/read/30079617/use-of-antipruritic-and-rehydrating-foams-on-localized-lesions-of-atopic-dermatitis-in-dogs-a-small-scale-pilot-and-comparative-double-blinded-study
#13
Emmanuel J Bensignor, Lionel J Fabriès
BACKGROUND: There is a lack of controlled studies evaluating the efficacy of topical nonsteroidal agents for the treatment of canine atopic dermatitis (cAD). HYPOTHESIS/OBJECTIVES: To compare the clinical efficacy of a commercial foam product (mousse), previously demonstrated to be effective in cAD (Foam A) with a foam/mousse containing components from plant extracts (Foam B). ANIMALS: Eight client-owned dogs with nonseasonal mild/moderate cAD...
August 5, 2018: Veterinary Dermatology
https://www.readbyqxmd.com/read/30075896/comparison-of-native-t1-strain-and-traditional-measures-of-cardiovascular-structure-and-function-by-cardiac-magnetic-resonance-imaging-in-patients-with-anderson-fabry-disease
#14
Hunter C Wilson, Stephanie Ambach, Peace C Madueme, Philip R Khoury, Robert J Hopkin, John L Jefferies
Cardiovascular magnetic resonance imaging (CMR) has emerged as a powerful tool to illuminate cardiovascular pathology in Anderson-Fabry disease (AFD); however, further study is required to develop clinically useful monitoring paradigms. The objective of this study was to retrospectively evaluate strain, native septal T1 values, and standard CMR measurements in a cohort of AFD patients to characterize useful measures of cardiovascular dysfunction that may be derived from a CMR platform. Eighteen patients were identified (n = 8 males) and divided according to presence or absence of left ventricular hypertrophy (LVH)...
June 22, 2018: American Journal of Cardiology
https://www.readbyqxmd.com/read/30072611/comparison-between-different-structures-of-suspended-core-microstructured-optical-fibers-for-volatiles-sensing
#15
Diego Lopez-Torres, Aitor Lopez-Aldaba, Cesar Elosua, Jean L Auguste, Rapahel Jamier, Philippe Roy, Manuel Lopez-Amo, Francisco J Arregui
In this paper, different core structures of microstructured optical fibers (MOFs) for low-finesse Fabry⁻Pérot (FP) sensors are experimentally compared to get the highest sensitivity. These devices are designed for volatile organic compounds (VOCs) measurements. Indium tin oxide (ITO) thin films were deposited by sputtering on the MOFs and different optical fast Fourier transform (FFT) phase responses from the FP were measured for saturated atmospheres of ethanol. It has been demonstrated that the sensitivities of the developed sensors depend strongly on the geometry and the dimensions of the MOF-cores...
August 2, 2018: Sensors
https://www.readbyqxmd.com/read/30066432/low-threshold-fabry-p%C3%A3-rot-mode-lasing-from-lead-iodide-trapezoidal-nanoplatelets
#16
Yangguang Zhong, Qi Wei, Zhen Liu, Qiuyu Shang, Liyun Zhao, Ruiwen Shao, Zhepeng Zhang, Jie Chen, Wenna Du, Chao Shen, Jun Zhang, Yanfeng Zhang, Peng Gao, Guichuan Xing, Xinfeng Liu, Qing Zhang
Lead Iodide (PbI2 ) is a layered semiconductor with direct band gap holding great promises in green light emission and detection devices. Recently, PbI2 planar lasers are demonstrated using hexagonal whispering-gallery-mode microcavities, but the lasing threshold is quite high. In this work, lasing from vapor phase deposition derived PbI2 trapezoidal nanoplatelets (NPs) with threshold that is at least an order of magnitude lower than the previous value is reported. The growth mechanism of the trapezoidal NPs is explored and attributed to the synergistic effects of van der Waals interactions and lattice mismatching...
July 31, 2018: Small
https://www.readbyqxmd.com/read/30064518/deep-characterization-of-the-anti-drug-antibodies-developed-in-fabry-disease-patients-a-prospective-analysis-from-the-french-multicenter-cohort-ffabry
#17
Wladimir Mauhin, Olivier Lidove, Damien Amelin, Foudil Lamari, Catherine Caillaud, Federico Mingozzi, Gaëlle Dzangué-Tchoupou, Louiza Arouche-Delaperche, Claire Douillard, Bertrand Dussol, Vanessa Leguy-Seguin, Pauline D'Halluin, Esther Noel, Thierry Zenone, Marie Matignon, François Maillot, Kim-Heang Ly, Gérard Besson, Marjolaine Willems, Fabien Labombarda, Agathe Masseau, Christian Lavigne, Roseline Froissart, Didier Lacombe, Jean Marc Ziza, Eric Hachulla, Olivier Benveniste
BACKGROUND: Fabry disease (OMIM #301500) is an X-linked disorder caused by alpha-galactosidase A deficiency with two major clinical phenotypes: classic and non-classic of different prognosis. From 2001, enzyme replacement therapies (ERT) have been available. We aimed to determine the epidemiology and the functional characteristics of anti-drug antibodies. Patients from the French multicenter cohort FFABRY (n = 103 patients, 53 males) were prospectively screened for total anti-agalsidase IgG and IgG subclasses with a home-made enzyme-linked immunosorbent assay (ELISA), enzyme-inhibition assessed with neutralization assays and lysoGb3 plasma levels, and compared for clinical outcomes...
July 31, 2018: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/30062314/progression-of-left-ventricular-fibrosis-in-a-woman-with-anderson-fabry-disease-longitudinal-observations-using-two-dimensional-speckle-tracking-echocardiography
#18
Yoshiyuki Takase, Tomoo Nagai, Takao Kubota, Hiroaki Takeo, Motomichi Kosuga, Torayuki Okuyama, Hirotsugu Tabata
No abstract text is available yet for this article.
April 2018: CASE: Cardiovascular Imaging Case Reports
https://www.readbyqxmd.com/read/30061722/rhythmicity-of-mood-symptoms-in-individuals-at-risk-for-psychiatric-disorders
#19
Luísa K Pilz, Alicia Carissimi, Melissa A B Oliveira, Ana Paula Francisco, Raul C Fabris, Madeleine S Medeiros, Marina Scop, Benicio N Frey, Ana Adan, Maria Paz Hidalgo
Despite emerging evidence that disruption in circadian rhythms may contribute to the pathophysiology of psychiatric disorders, there is a significant knowledge gap on the rhythmicity of psychological symptoms. Here, we aimed at investigating the rhythmicity of mood symptoms in individuals at risk for psychiatric disorders. 391 Brazilian and 317 Spanish participants completed the Self-Reporting Questionnaire-20 for non-psychotic mental disorders; the Mood Rhythm Instrument was used to assess rhythmicity of mood symptoms and the Munich ChronoType Questionnaire to assess sleep patterns...
July 30, 2018: Scientific Reports
https://www.readbyqxmd.com/read/30061658/enhanced-water-splitting-under-modal-strong-coupling-conditions
#20
Xu Shi, Kosei Ueno, Tomoya Oshikiri, Quan Sun, Keiji Sasaki, Hiroaki Misawa
Strong coupling between plasmons and optical modes, such as waveguide or resonator modes, gives rise to a splitting in the plasmon absorption band. As a result, two new hybrid modes are formed that exhibit near-field enhancement effects. These hybrid modes have been exploited to improve light absorption in a number of systems. Here we show that this modal strong coupling between a Fabry-Pérot nanocavity mode and a localized surface plasmon resonance (LSPR) facilitates water splitting reactions. We use a gold nanoparticle (Au-NP)/TiO2 /Au-film structure as a photoanode...
July 30, 2018: Nature Nanotechnology
keyword
keyword
165635
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"