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Kassiani Kytidou, Jules Beekwilder, Marta Artola, Eline van Meel, Ruud H P Wilbers, Geri F Moolenaar, Nora Goosen, Maria J Ferraz, Rebecca Katzy, Patrick Voskamp, Bogdan I Florea, Cornelis H Hokke, Herman S Overkleeft, Arjen Schots, Dirk Bosch, Navraj Pannu, Johannes M F G Aerts
α-Galactosidases (EC are retaining glycosidases that cleave terminal α-linked galactose residues from glycoconjugate substrates. α-Galactosidases take part in the turnover of cell wall-associated galactomannans in plants and in the lysosomal degradation of glycosphingolipids in animals. Deficiency of human α-galactosidase A (α-Gal A) causes Fabry disease (FD), a heritable, X-linked lysosomal storage disorder, characterized by accumulation of globotriaosylceramide (Gb3) and globotriaosylsphingosine (lysoGb3)...
April 19, 2018: Journal of Biological Chemistry
H Ahmadzadehfar, P Albers, A Bockisch, M Boegemann, C Böhme, W Burchert, M Dietlein, A Drzezga, U Fabry, G Feldmann, A Heidenreich, A Heinzel, K Herrmann, A Heyll, C Höhling, C Kreuzer, D Laufer, R Mengel, F M Mottaghy, H-W Müller, S C Müller, E Ost, K Rahbar, W Reifenhäuser, M Schäfers, C Schlenkhoff, M Schmidt, I Schmidt-Wolf, C Wildenhain, B Zimmer, M Essler
In the last 3 years, Lutetium-177 prostate-specific membrane antigen radioligand therapy (Lu-177-PSMA-RLT) has received increasing attention in nuclear medicine as a new form of treatment for castration-resistant metastatic prostate cancer. This therapy combines the radionuclide Lutetium-177, which has been therapeutically used in nuclear medicine for many years, with a molecular target of the transmembrane prostate-specific membrane antigen expressed by prostate cancer cells. Since there are no prospective randomized studies on Lu-177-PSMA-RLT and the question of reimbursement has repeatedly been the subject of review by the MDK Nordrhein (Medischenische Dienst der Krankenversicherung), there was a desire because of the increasing number of patients being treated to clarify under which circumstances Lu-177-PSMA-RLT can be reimbursed by German statutory health insurance...
April 18, 2018: Der Urologe. Ausg. A
Diletta Arcidiacono, Arben Dedja, Cinzia Giacometti, Matteo Fassan, Daniele Nucci, Simona Francia, Federico Fabris, Alice Zaramella, Emily J Gallagher, Mauro Cassaro, Massimo Rugge, Derek LeRoith, Alfredo Alberti, Stefano Realdon
Hyperinsulinemia could have a role in the growing incidence of esophageal adenocarcinoma (EAC) and its pre-cancerous lesion, Barrett's Esophagus, a possible consequence of Gastro-Esophageal Reflux Disease. Obesity is known to mediate esophageal carcinogenesis through different mechanisms including insulin-resistance leading to hyperinsulinemia, which may mediate cancer progression via the insulin/insulin-like growth factor axis. We used the hyperinsulinemic non-obese FVB/N (Friend leukemia virus B strain) MKR (muscle (M)-IGF1R-lysine (K)-arginine (R) mouse model to evaluate the exclusive role of hyperinsulinemia in the pathogenesis of EAC related to duodeno-esophageal reflux...
April 14, 2018: International Journal of Molecular Sciences
(no author information available yet)
No abstract text is available yet for this article.
April 16, 2018: Journal of Medical Genetics
Po Phyu, Aine Merwick, Indran Davagnanam, Fay Bolsover, Fatima Jichi, Claudia Wheeler-Kingshott, Xavier Golay, Deralynn Hughes, Lisa Cipolotti, Elaine Murphy, Robin H Lachmann, David John Werring
OBJECTIVE: To assess resting cerebral blood flow (CBF) in the whole-brain and cerebral white matter (WM) and gray matter (GM) of adults with Fabry disease (FD), using arterial spin labeling (ASL) MRI, and to investigate CBF correlations with WM hyperintensity (WMH) volume and the circulating biomarker lyso-Gb3. METHODS: This cross-sectional, case-control study included 25 patients with genetically confirmed FD and 18 age-matched healthy controls. We quantified resting CBF using Quantitative Signal Targeting With Alternating Radiofrequency Labeling of Arterial Regions (QUASAR) ASL MRI...
April 17, 2018: Neurology
Anne-Katrin Giese, Natalia S Rost
No abstract text is available yet for this article.
April 17, 2018: Neurology
M López Rodríguez
Fabry disease is an X-linked inborn disease caused by deficit of alpha-galactosidaseA. This results in accumulation of glycosphingolipids in all cells and tissues. All males should receive enzyme replacement treatment in case of very low or undetectable levels of alpha-galactosidaseA. Female carriers and males with marginally levels of alpha-galactosidaseA should be treated in case of renal, neurologic o cardiac manifestations. There are two intravenous formulations of human recombinant enzyme, agalsidase alpha and agalsidase beta, showing similar efficacy and safety...
April 13, 2018: Revista Clínica Española
Paula Marchesini, Alan Franco Barbosa, Cristiane Franco, Tatiane Novato, Mirza Nalesso Gomes Sanches, Mário Geraldo de Carvalho, Rodrigo Luiz Fabri, Erik Daemon, Caio Márcio Oliveira Monteiro
This study evaluated the acaricidal activity of the methanol extract of Acmella oleracea with 0.187% of spilanthol against immature stages of Amblyomma sculptum. The packet test was used to evaluate the extract's activity on unengorged larvae and nymphs, testing concentrations of 0.4 to 50 mg/mL for larvae and 12.5 to 200.0 mg/mL for nymphs. For the engorged stages, the immersion test was used, at concentrations of 0.4 to 50 mg/mL for larvae and 12.5 to 200.0 mg/mL for nymphs. The methanol extract caused 100% mortality of the unengorged larvae and nymphs starting at concentrations of 12...
April 30, 2018: Veterinary Parasitology
Yi Lin, Yuyun Lu, Zhixuan Song, Dejian Huang
The acorns of Quercus fabri Hance tree, or the mandarin duck fruits have been consumed by locals in the form of noodle, yet there is little scientific study. We found that they have much lower digestibility compared with that of rice flour. Solvent extraction using hexane, dichloromethane, and acetone-ethanol-water (AEW) mixture yielded three fractions but the starch hydrolase inhibitors were only found in the AEW fraction. Using assay-guided fractionation of the extracts, we were able to further separate the active compounds by using Sephadex LH-20 column and characterize the inhibitor chemical identities by LC-MS( n ) ...
August 30, 2018: Food Chemistry
Juliana Gonçalves Pires, Sara Salustiano Zabini, Aline Silva Braga, Rita de Cássia Fabris, Flaviana Bombarda de Andrade, Rodrigo Cardoso de Oliveira, Ana Carolina Magalhães
OBJECTIVES: This study evaluated the effect of the hydroalcoholic extracts of Myracrodruon urundeuva All. and Qualea grandiflora Mart. leaves (alone or combined) on the viability of Streptococcus mutans biofilm and on the prevention of enamel demineralization. METHODS: Strain of S. mutans (ATCC 21175) was reactivated in BHI broth. Minimum inhibitory concentration, minimum bactericidal concentration, minimum inhibition biofilm concentration and minimum eradication biofilm concentration were determined in order to choose the concentrations to be tested under biofilm model...
April 6, 2018: Archives of Oral Biology
Dominique P Germain, Eva Brand, Alessandro Burlina, Franco Cecchi, Scott C Garman, Judy Kempf, Dawn A Laney, Aleš Linhart, László Maródi, Kathy Nicholls, Alberto Ortiz, Federico Pieruzzi, Suma P Shankar, Stephen Waldek, Christoph Wanner, Ana Jovanovic
BACKGROUND: The p.Asn215Ser or p.N215S GLA variant has been associated with late-onset cardiac variant of Fabry disease. METHODS: To expand on the scarce phenotype data, we analyzed natural history data from 125 p.N215S patients (66 females, 59 males) enrolled in the Fabry Registry (NCT00196742) and compared it with data from 401 patients (237 females, 164 males) harboring mutations associated with classic Fabry disease. We evaluated interventricular septum thickness (IVST), left ventricular posterior wall thickness (LVPWT), estimated glomerular filtration rate and severe clinical events...
April 12, 2018: Molecular Genetics & Genomic Medicine
Weifang Zhang, Yingwu Li, Bo Jin, Feifei Ren, Hongxun Wang, Wei Dai
A Fiber Bragg Grating (FBG) interrogation system with a self-adaption threshold peak detection algorithm is proposed and experimentally demonstrated in this study. This system is composed of a field programmable gate array (FPGA) and advanced RISC machine (ARM) platform, tunable Fabry-Perot (F-P) filter and optical switch. To improve system resolution, the F-P filter was employed. As this filter is non-linear, this causes the shifting of central wavelengths with the deviation compensated by the parts of the circuit...
April 8, 2018: Sensors
Letizia Spinelli, Massimo Imbriaco, Carmela Nappi, Emanuele Nicolai, Giuseppe Giugliano, Andrea Ponsiglione, Tommaso Claudio Diomiaiuti, Eleonora Riccio, Giovanni Duro, Antonio Pisani, Bruno Trimarco, Alberto Cuocolo
BACKGROUND: Hybrid18 F-fluorodeoxyglucose (FDG) positron emission tomography and magnetic resonance imaging may differentiate mature fibrosis or scar from fibrosis associated to active inflammation in patients with Anderson-Fabry disease, even in nonhypertrophic stage. This study was designed to compare the results of positron emission tomography and magnetic resonance cardiac imaging with those of speckle-tracking echocardiography in heterozygous Anderson-Fabry disease females. METHODS AND RESULTS: Twenty-four heterozygous females carrying α-galactosidase A mutation and without left ventricular hypertrophy underwent cardiac positron emission tomography and magnetic resonance using18 F-FDG for glucose uptake and 2-dimensional strain echocardiography...
April 2018: Circulation. Cardiovascular Imaging
L Lavalle, A S Thomas, B Beaton, H Ebrahim, M Reed, U Ramaswami, P Elliott, A B Mehta, D A Hughes
BACKGROUND: Fabry disease (FD) results from X-linked inheritance of a mutation in the GLA gene, encoding for alpha galactosidase A, and is characterized by heterogeneous clinical manifestations. Two phenotypes have been described "Classic" and "late onset" which cannot be predicted exclusively by genotype. The latter has been considered an attenuated form of the disease often affecting a single organ system commonly the heart. Recent studies have demonstrated that cardiac outcomes are similar in patients with classic and late onset mutations...
2018: PloS One
Irene Simonetta, Antonino Tuttolomondo, Tiziana Di Chiara, Salvatore Miceli
Fabry's disease is a genetic disorder of X-linked inheritance caused by mutations in the alpha galactosidase A gene resulting in deficiency of this lysosomal enzyme. The progressive accumulation of glycosphingolipids, caused by the inadequate enzymatic activity, is responsible of organ dysfunction and thus of clinical manifestations. In presence of a high clinical suspicion, a careful physical examination and specific laboratory tests are required, finally diagnosis of Fabry's disease is confirmed by demonstration of absence or reduced alpha galactosidase A enzyme activity in hemizygous men and gene typing in heterozygous females; in fact the performance of enzymatic activity assay alone in women is inconclusive...
April 4, 2018: Current Gene Therapy
Sergio Fabris, David A MacLean
Skeletal muscle (SM) health and integrity is dependent on the dynamic balance between protein synthesis and degradation, and central to this process is the availability of amino acids (AA) in the amino pool. While Doxorubicin (DOX) remains one of the most widely used chemotherapeutic agents for the treatment of solid and hematological malignancies, little is known of the effect of the drug on SM, particularly its effect on the availability of amino acids in the tissue. The purpose of this study was to examine the effect of DOX administration on vascular, interstitial and intracellular concentrations of AA in SM of the rat up to 8 days after the administration of a 1...
2018: PloS One
Daisuke Inoue, Daehwan Jung, Justin Norman, Yating Wan, Nobuhiko Nishiyama, Shigehisa Arai, Arthur C Gossard, John E Bowers
We report the first demonstration of direct modulation of InAs/GaAs quantum dot (QD) lasers grown on on-axis (001) Si substrate. A low threading dislocation density GaAs buffer layer enables us to grow a high quality 5-layered QD active region on on-axis Si substrate. The active layer has p-modulation doped GaAs barrier layers with a hole concentration of 5 × 1017 cm-3 to suppress gain saturation. Small-signal measurement on a 3 × 580 μm2 Fabry-Perot laser showed a 3dB bandwidth of 6.5 GHz at a bias current of 116 mA...
March 19, 2018: Optics Express
Matteo Lucchini, Giacinto D Lucarelli, Mario Murari, Andrea Trabattoni, Nicola Fabris, Fabio Frassetto, Sandro De Silvestri, Luca Poletto, Mauro Nisoli
Sub-10-fs pulses tunable in the extreme-ultraviolet (XUV) spectral region are particularly important in many research fields: from atomic and molecular spectroscopy to the study of relaxation processes in solids and transition phase processes, from holography to free-electron laser injection. A crucial prerequisite for all applications is the accurate measurement of the temporal characteristics of these pulses. To fulfill this purpose, many phase retrieval algorithms have been successfully applied to reconstruct XUV attosecond pulses...
March 19, 2018: Optics Express
Badreddine Ratni, Jianjia Yi, Xumin Ding, André de Lustrac, Kuang Zhang, Gérard-Pascal Piau, Shah Nawaz Burokur
A metal-dielectric-metal gradient phase partially reflecting surface based on the combination of a gradient index dielectric substrate with an inductive and a capacitive grids, is designed at microwave frequencies for antenna applications. The gradient index is obtained by realizing air holes of different dimensions in a dielectric host material. A prototype of the gradient index dielectric substrate is fabricated through three-dimensional printing, an additive fabrication technology. It is then associated to two patterned metallic grids to realize a partially reflecting surface with a gradient phase behavior...
March 19, 2018: Optics Express
S Gundogdu, H S Pisheh, A Demir, M Gunoven, A Aydinli, C Sirtori
Temperature rise during operation is a central concern of semiconductor lasers and especially difficult to measure during a pulsed operation. We present a technique for in situ time-resolved temperature measurement of quantum cascade lasers operating in a pulsed mode at ~9.25 μm emission wavelength. Using a step-scan approach with 5 ns resolution, we measure the temporal evolution of the spectral density, observing longitudinal Fabry-Perot modes that correspond to different transverse modes. Considering the multiple thin layers that make up the active layer and the associated Kapitza resistance, thermal properties of QCLs are significantly different than bulk-like laser diodes where this approach was successfully used...
March 19, 2018: Optics Express
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