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https://www.readbyqxmd.com/read/28813096/cytotoxicity-and-bacterial-membrane-destabilization-induced-by-annona-squamosa-l-extracts
#1
Nícolas C C Pinto, Jucélia B Silva, Laura M Menegati, Maria Clara M R Guedes, Lucas B Marques, Thiago P DA Silva, Rossana C N DE Melo, Elaine M DE Souza-Fagundes, Marcos J Salvador, Elita Scio, Rodrigo L Fabri
This study aimed to further investigate the cytotoxicity against tumor cell lines and several bacterial strains of Annona squamosa and its mode of action. Methanol extracts of A. squamosa leaves (ASL) and seeds (ASS) were used. ASL showed significant antibacterial activity against S. aureus, K. pneumoniae and E. faecalis with MIC values of 78, 78 and 39 µg/mL respectively. Moreover, ASL exhibited significant biofilm disruption, rapid time dependent kinetics of bacterial killing, increased membrane permeability and significantly reduced the cell numbers and viability...
August 14, 2017: Anais da Academia Brasileira de Ciências
https://www.readbyqxmd.com/read/28809920/single-longitudinal-mode-broadband-tunable-random-laser
#2
Heba Shawki, Hussein Kotb, Diaa Khalil
In this work, we demonstrate a broadband tunable single-longitudinal-mode (SLM) random laser based on Rayleigh backscattering in a standard single-mode fiber. The wide tuning range of this SLM fiber laser over 1500-1570 nm is demonstrated with a linewidth of 4.5-30 kHz. The tuning is achieved using a tunable bandpass Fabry-Perot filter, and a semiconductor optical amplifier is used as the wide-bandwidth gain medium. The laser is able to operate in the S + C + L band.
August 15, 2017: Optics Letters
https://www.readbyqxmd.com/read/28809894/high-sensitivity-and-large-dynamic-range-refractive-index-sensors-employing-weak-composite-fabry-perot-cavities
#3
Pengcheng Chen, Xuewen Shu, Haoran Cao, Kate Sugden
Most sensors face a common trade-off between high sensitivity and a large dynamic range. We demonstrate here an all-fiber refractometer based on a dual-cavity Fabry-Perot interferometer (FPI) that possesses the advantage of both high sensitivity and a large dynamic range. Since the two composite cavities have a large cavity length difference, one can observe both fine and coarse fringes, which correspond to the long cavity and the short cavity, respectively. The short-cavity FPI and the use of an intensity demodulation method mean that the individual fine fringe dips correspond to a series of quasi-continuous highly sensitive zones for refractive index measurement...
August 15, 2017: Optics Letters
https://www.readbyqxmd.com/read/28809101/a-microsphere-supported-lipid-bilayer-platform-for-dna-reactions-on-a-fluid-surface
#4
Aurora Fabry-Wood, Madalyn Elise Fetrow, Carl W Brown Iii, Nicholas A Baker, Nadiezda Fernandez Oropeza, Andrew P Shreve, Gabriel A Montano, Darko Stefanovic, Matthew R Lakin, Steven W Graves
We report a versatile microsphere-supported lipid bilayer system that can serve as a general-purpose platform for implementing DNA nanotechnologies on a fluid surface. To demonstrate our platform, we implemented both toehold-mediated strand displacement (TMSD) and DNAzyme reactions, which are typically performed in solution and which are the cornerstone of DNA-based molecular logic and dynamic DNA nanotechnology, on the surface. We functionalized microspheres bearing supported lipid bilayers (µSLBs) with membrane-bound nucleic acid components...
August 15, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/28808181/screening-of-fabry-disease-in-patients-with-end-stage-renal-disease-of-unknown-etiology-the-first-thailand-study
#5
Objoon Trachoo, Paisan Jittorntam, Sarunpong Pibalyart, Saowanee Kajanachumphol, Norasak Suvachittanont, Suthep Patputthipong, Piyatida Chuengsaman, Arkom Nongnuch
We aimed to explore the prevalence of Fabry disease in Thai patients who were diagnosed with end-stage renal disease (ESRD) of an unknown origin. Venous blood samples were collected from ESRD patients for biochemical and molecular studies. Alpha-galactosidase A (α-GAL A) screening was performed from dried-blood spots using fluorometry. Molecular confirmation was performed using DNA sequencing of the GLA gene. A total of 142 male and female patients were included in this study. Ten patients (7.04%) exhibited a significant decrease in α-GAL A activity...
October 17, 2016: Journal of Biomedical Research
https://www.readbyqxmd.com/read/28799081/the-psychosocial-impact-of-carrying-a-debated-variant-in-the-gla-gene
#6
Sarah Macklin, Dawn Laney, Emily Lisi, Andrea Atherton, Elizabeth Smith
The clinical significance of the c.427G>A (p.A143T) variant in GLA is a topic of debate within the lysosomal storage disease community. A review of the literature and published case reports found the clinical impact of the variant to range from classic Fabry symptoms to healthy unaffected males with normal alpha- galactosidase enzyme levels, leaving clinicians unsure of how to manage these individuals. As the number of states testing for Fabry disease on their newborn screening panel has increased, more people with this variant are being identified...
August 10, 2017: Journal of Genetic Counseling
https://www.readbyqxmd.com/read/28798027/fabry-disease-a-rare-condition-emerging-from-the-darkness
#7
EDITORIAL
Perry M Elliott
No abstract text is available yet for this article.
August 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28798024/fabry-disease-in-families-with-hypertrophic-cardiomyopathy-clinical-manifestations-in-the-classic-and-later-onset-phenotypes
#8
Berglind Adalsteinsdottir, Runolfur Palsson, Robert J Desnick, Marianna Gardarsdottir, Polakit Teekakirikul, Martin Maron, Evan Appelbaum, Ulf Neisius, Barry J Maron, Michael A Burke, Brenden Chen, Silvere Pagant, Christoffer V Madsen, Ragnar Danielsen, Reynir Arngrimsson, Ulla Feldt-Rasmussen, Jonathan G Seidman, Christine E Seidman, Gunnar Th Gunnarsson
BACKGROUND: The screening of Icelandic patients clinically diagnosed with hypertrophic cardiomyopathy resulted in identification of 8 individuals from 2 families with X-linked Fabry disease (FD) caused by GLA(α-galactosidase A gene) mutations encoding p.D322E (family A) or p.I232T (family B). METHODS AND RESULTS: Familial screening of at-risk relatives identified mutations in 16 family A members (8 men and 8 heterozygotes) and 25 family B members (10 men and 15 heterozygotes)...
August 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28797308/coeliac-disease-in-infants-antibodies-to-deamidated-gliadin-peptide-come-first
#9
Michele Arigliani, Francesca Rech Morassutti, Martina Fabris, Paola Melli, Elio Tonutti, Paola Cogo
BACKGROUND: The onset of coeliac disease (CD) in the first year of life is uncommon and the diagnosis can be challenging due to the suboptimal sensitivity of tissue transglutaminase antibodies (tTG) at this age and the many other possible causes of malabsorption in infants. Antibodies to deamidated gliadin peptides (anti-DGPs), especially IgG, may appear earlier than IgA anti-tTG in very young children with CD. CASE PRESENTATION: We report here on an 8-month-old child who was evaluated for failure to thrive, constipation and developmental delay...
August 10, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28794969/an-unusual-type-of-myeloid-sarcoma-localization-following-myelofibrosis-a-case-report-and-literature-review
#10
Nicola Orofino, Daniele Cattaneo, Cristina Bucelli, Loredana Pettine, Sonia Fabris, Umberto Gianelli, Nicola Stefano Fracchiolla, Agostino Cortelezzi, Alessandra Iurlo
Myeloid Sarcoma (MS) is a rare malignancy that can present as an isolated disease or more frequently in association with or following acute myeloid leukemia or other myeloid neoplasms and rarely following myelofibrosis. Since molecular pathogenesis and prognostic factors of MS are not well understood, its prognosis remains poor even in the era of novel agents and target therapies. We report the case of a patient with MS following myelofibrosis with multiple subcutaneous, cutaneous and muscle localizations; the latter has been reported in the literature as anecdotal...
2017: Leukemia Research Reports
https://www.readbyqxmd.com/read/28794847/neuro-otological-and-peripheral-nerve-involvement-in-fabry-disease
#11
Sergio Carmona, Romina Weinschelbaum, Ana Pardal, Cintia Marchesoni, Paz Zuberbuhler, Patricia Acosta, Guillermo Cáceres, Isaac Kisinovsky, Luciana Bayón, Ricardo Reisin
Fabry disease (FD) is an X-linked lysosomal storage disease, with multisystemic glycosphingolipids deposits. Neuro-otological involvement leading to hearing loss and vestibular dysfunctions has been described, but there is limited information about the frequency, site of lesion, or the relationship with peripheral neuropathy. The aim was to evaluate the presence of auditory and vestibular symptoms, and assess neurophysiological involvement of the VIII cranial nerve, correlating these findings with clinical and neurophysiological features of peripheral neuropathy...
July 18, 2017: Audiology Research
https://www.readbyqxmd.com/read/28793143/intraoperative-diagnosis-of-anderson-fabry-disease-in-patients-with-obstructive-hypertrophic-cardiomyopathy-undergoing-surgical-myectomy
#12
Franco Cecchi, Maria Iascone, Niccolò Maurizi, Laura Pezzoli, Irene Binaco, Elena Biagini, Maria Laura Fibbi, Iacopo Olivotto, Federico Pieruzzi, Ana Fruntelata, Lucian Dorobantu, Claudio Rapezzi, Paolo Ferrazzi
Importance: Diagnostic screening for Anderson-Fabry cardiomyopathy (AFC) is performed in the presence of specific clinical red flags in patients with hypertrophic cardiomyopathy (HCM) older than 25 years. However, left ventricular outflow tract obstruction (LVOTO) has been traditionally considered an exclusion criteria for AFC. Objective: To examine a series of patients diagnosed with HCM and severe basal LVOTO undergoing myectomy in whom the diagnosis of AFC was suspected by the cardiac surgeon intraoperatively and confirmed by histological and genetic examinations...
August 9, 2017: JAMA Cardiology
https://www.readbyqxmd.com/read/28783313/electrically-tunable-dye-emission-via-microcavity-integrated-pdms-gel-actuator
#13
Markus Franke, Irma Slowik, Philipp Jan Mehner, Georgi Paschew, Andreas Voigt, Hartmut Fröb, Karl Leo, Andreas Richter
Electrically tunable microcavities are essential elements for tunable laser sources indispensable for modern telecommunication and spectroscopy. However, most device concepts suffer from extensive lithography or etching for membrane processing. Here, we present an electrically and continuously tunable, multi half-wavelength microcavity with a quality factor > 1000 as an easy-to-fabricate platform with potential use for vertical-cavity surface-emitting lasers. The microcavity has a Fabry-Pérot structure consisting of ultra-soft PDMS gel with a thickness of 14 - 15 µm and capped by a distributed Bragg reflector on the bottom end and a silver layer serving as top mirror and electrode...
August 7, 2017: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/28775215/observation-of-coherent-elastic-neutrino-nucleus-scattering
#14
D Akimov, J B Albert, P An, C Awe, P S Barbeau, B Becker, V Belov, A Brown, A Bolozdynya, B Cabrera-Palmer, M Cervantes, J I Collar, R J Cooper, R L Cooper, C Cuesta, D J Dean, J A Detwiler, A Eberhardt, Y Efremenko, S R Elliott, E M Erkela, L Fabris, M Febbraro, N E Fields, W Fox, Z Fu, A Galindo-Uribarri, M P Green, M Hai, M R Heath, S Hedges, D Hornback, T W Hossbach, E B Iverson, L J Kaufman, S Ki, S R Klein, A Khromov, A Konovalov, M Kremer, A Kumpan, C Leadbetter, L Li, W Lu, K Mann, D M Markoff, K Miller, H Moreno, P E Mueller, J Newby, J L Orrell, C T Overman, D S Parno, S Penttila, G Perumpilly, H Ray, J Raybern, D Reyna, G C Rich, D Rimal, D Rudik, K Scholberg, B J Scholz, G Sinev, W M Snow, V Sosnovtsev, A Shakirov, S Suchyta, B Suh, R Tayloe, R T Thornton, I Tolstukhin, J Vanderwerp, R L Varner, C J Virtue, Z Wan, J Yoo, C-H Yu, A Zawada, J Zettlemoyer, A M Zderic
The coherent elastic scattering of neutrinos off nuclei has eluded detection for four decades, even though its predicted cross-section is the largest by far of all low-energy neutrino couplings. This mode of interaction provides new opportunities to study neutrino properties, and leads to a miniaturization of detector size, with potential technological applications. We observe this process at a 6.7-sigma confidence level, using a low-background, 14.6-kg CsI[Na] scintillator exposed to the neutrino emissions from the Spallation Neutron Source (SNS) at Oak Ridge National Laboratory...
August 3, 2017: Science
https://www.readbyqxmd.com/read/28774197/thrombotic-and-hemorrhagic-conditions-due-to-a-gain-of-function-of-coagulation-proteins-a-special-type-of-clotting-disorders
#15
Antonio Girolami, Elisabetta Cosi, Silvia Ferrari, Annamaria Lombardi, Fabrizio Fabris
Coagulation disorders can be classified into 2 types, namely, type I and type II. In the former, there is a concomitant decrease in factor activity and antigen (activity-antigen ratio is 1), whereas in the latter, there is a discrepancy between factor activity which is always low and antigen which is normal or near normal (activity-antigen ratio is <1, eg, 0.5). Recently, several gain-of-function disorders have been described. These are characterized by an increased activity with respect to the antigen level...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28769867/changes-in-ionic-conductance-signature-of-nociceptive-neurons-underlying-fabry-disease-phenotype
#16
Barbara Namer, Kirstin Ørstavik, Roland Schmidt, Norbert Mair, Inge Petter Kleggetveit, Maximillian Zeidler, Theresa Martha, Ellen Jorum, Martin Schmelz, Theodora Kalpachidou, Michaela Kress, Michiel Langeslag
The first symptom arising in many Fabry patients is neuropathic pain due to changes in small myelinated and unmyelinated fibers in the periphery, which is subsequently followed by a loss of sensory perception. Here we studied changes in the peripheral nervous system of Fabry patients and a Fabry mouse model induced by deletion of α-galactosidase A (Gla(-/0)). The skin innervation of Gla(-/0) mice resembles that of the human Fabry patients. In Fabry diseased humans and Gla(-/0) mice, we observed similar sensory abnormalities, which were also observed in nerve fiber recordings in both patients and mice...
2017: Frontiers in Neurology
https://www.readbyqxmd.com/read/28768754/previously-unreported-in-women-galactosidase-alpha-pro409ser-variant-is-associated-with-fabry-disease
#17
Sushil Allen Luis, Joseph J Maleszewski, Phillip M Young, Hartzell V Schaff, Naveen L Pereira
No abstract text is available yet for this article.
August 2017: Circulation. Cardiovascular Genetics
https://www.readbyqxmd.com/read/28767657/maternal-ptsd-and-corresponding-neural-activity-mediate-effects-of-child-exposure-to-violence-on-child-ptsd-symptoms
#18
Daniel S Schechter, Dominik A Moser, Tatjana Aue, Marianne Gex-Fabry, Virginie C Pointet, Maria I Cordero, Francesca Suardi, Aurelia Manini, Marylène Vital, Ana Sancho Rossignol, Molly Rothenberg, Alexandre G Dayer, Francois Ansermet, Sandra Rusconi Serpa
The aim of this study was to examine the relationship of maternal interpersonal violence-related posttraumatic stress disorder (IPV-PTSD), associated neural activity in response to mother-child relational stimuli, and child psychopathology indicators at child ages 12-42 months and one year later. The study tested the hypothesis that decreased maternal neural activity in regions that subserve emotion regulation would be associated with child symptoms associated with emotional dysregulation at both time points...
2017: PloS One
https://www.readbyqxmd.com/read/28765613/structural-and-functional-identification-of-vasculogenic-mimicry-in-vitro
#19
Dusan Racordon, Andrés Valdivia, Gabriel Mingo, Rafaela Erices, Raúl Aravena, Felice Santoro, Maria Loreto Bravo, Carolina Ramirez, Pamela Gonzalez, Alejandra Sandoval, Alfonso González, Claudio Retamal, Marcelo J Kogan, Sumie Kato, Mauricio A Cuello, German Osorio, Francisco Nualart, Pedro Alvares, Araceli Gago-Arias, Daniella Fabri, Ignacio Espinoza, Beatriz Sanchez, Alejandro H Corvalán, Mauricio P Pinto, Gareth I Owen
Vasculogenic mimicry (VM) describes a process by which cancer cells establish an alternative perfusion pathway in an endothelial cell-free manner. Despite its strong correlation with reduced patient survival, controversy still surrounds the existence of an in vitro model of VM. Furthermore, many studies that claim to demonstrate VM fail to provide solid evidence of true hollow channels, raising concerns as to whether actual VM is actually being examined. Herein, we provide a standardized in vitro assay that recreates the formation of functional hollow channels using ovarian cancer cell lines, cancer spheres and primary cultures derived from ovarian cancer ascites...
August 1, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28763515/retrospective-study-of-long-term-outcomes-of-enzyme-replacement-therapy-in-fabry-disease-analysis-of-prognostic-factors
#20
Maarten Arends, Marieke Biegstraaten, Derralynn A Hughes, Atul Mehta, Perry M Elliott, Daniel Oder, Oliver T Watkinson, Frédéric M Vaz, André B P van Kuilenburg, Christoph Wanner, Carla E M Hollak
Despite enzyme replacement therapy, disease progression is observed in patients with Fabry disease. Identification of factors that predict disease progression is needed to refine guidelines on initiation and cessation of enzyme replacement therapy. To study the association of potential biochemical and clinical prognostic factors with the disease course (clinical events, progression of cardiac and renal disease) we retrospectively evaluated 293 treated patients from three international centers of excellence...
2017: PloS One
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