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JOURNAL ARTICLE
Maria Carmela Pera, Marco Luigetti, Serena Sivo, Leonardo Lapenta, Giuseppe Granata, Laura Antonaci, Giorgia Coratti, Nicola Forcina, Marika Pane, Eugenio Mercuri
No abstract text is available yet for this article.
October 2018: Neuromuscular Disorders: NMD
#22
JOURNAL ARTICLE
Gaetano Malgieri, Gianluca D'Abrosca, Luciano Pirone, Angelo Toto, Maddalena Palmieri, Luigi Russo, Michele Francesco Maria Sciacca, Rosarita Tatè, Valeria Sivo, Ilaria Baglivo, Roksana Majewska, Massimo Coletta, Paolo Vincenzo Pedone, Carla Isernia, Mario De Stefano, Stefano Gianni, Emilia Maria Pedone, Danilo Milardi, Roberto Fattorusso
Significant advances in the understanding of the molecular determinants of fibrillogenesis can be expected from comparative studies of the aggregation propensities of proteins with highly homologous structures but different folding pathways. Here, we fully characterize, by means of stopped-flow, T-jump, CD and DSC experiments, the unfolding mechanisms of three highly homologous proteins, zinc binding Ros87 and Ml153-149 and zinc-lacking Ml452-151 . The results indicate that the three proteins significantly differ in terms of stability and (un)folding mechanisms...
April 7, 2018: Chemical Science
#23
JOURNAL ARTICLE
Christina Ziemann, Alberto Escrig, Giuliana Bonvicini, Maria Jesús Ibáñez, Eliseo Monfort, Arturo Salomoni, Otto Creutzenberg
The exposure to respirable crystalline silica (RCS), e.g. quartz, in industrial settings can induce silicosis and may cause tumours in chronic periods. Consequently, RCS in the form of quartz and cristobalite has been classified as human lung carcinogen category 1 by the International Agency for Research on Cancer in 1997, acknowledging differences in hazardous potential depending on source as well as chemical, thermal, and mechanical history. The physico-chemical determinants of quartz toxicity are well understood and are linked to density and abundance of surface silanol groups/radicals...
May 1, 2017: Annals of Work Exposures and Health
#24
MULTICENTER STUDY
Maria C Pera, Domenico M Romeo, Alessandra Graziano, Concetta Palermo, Sonia Messina, Giovanni Baranello, Giorgia Coratti, Maria Massaro, Serena Sivo, Maria Teresa Arnoldi, Elena S Mazzone, Laura Antonaci, Leonardo Lapenta, Emilio Albamonte, Lavinia Fanelli, Roberto de Sanctis, Gian Luca Vita, Maria Sframeli, Marika Pane, Eugenio Mercuri
OBJECTIVE: To estimate the frequency of sleep disorders in young persons with type 2 and type 3 spinal muscular atrophy (SMA), and to evaluate the relationship between sleep disorders and different variables such as motor impairment, age, use of ventilation, and use of night orthoses. METHODS: A total of 85 young persons (6-25 years of age) with type 2 and type 3 SMA were assessed using the Sleep Disturbance Scale for Children (SDSC), a scale assessing different sleep factors, and the Hammersmith Functional Motor Scale Expanded (HFMSE), a scale evaluating motor impairment...
February 2017: Sleep Medicine
#25
JOURNAL ARTICLE
I Kontuľ, M Ješkovský, J Kaizer, A Šivo, M Richtáriková, P P Povinec, P Čech, P Steier, R Golser
Radiocarbon measurements of tree-ring samples collected in Vysoká pri Morave were compared with tree-ring data of the Žlkovce monitoring station situated 5km south-east from the Jaslovské Bohunice Nuclear Power Plant (NPP). Radiocarbon concentrations in Vysoká pri Morave and in Žlkovce tree rings were decreasing exponentially with decay constants of 14.48±1.23 y and 17.96±1.97 y, respectively, in agreement with similar results obtained at other radiocarbon stations. The Suess effect, represented by a dilution in 14 C levels by fossil fuel CO2 emissions, was observed in both tree-ring data sets...
August 2017: Applied Radiation and Isotopes
#26
JOURNAL ARTICLE
Alessandro Domenici, Anna Giuliani, Francesca Sivo, Clorinda Falcone, Giorgio Punzo, Paolo Menè
This study compares 5 different tidal automated peritoneal dialysis (APD) prescriptions. Six low-average and 6 high-average transporters performed 3 separate sessions with 5 different schedules: (A) 50% tidal with initial fill volume (FV) 2 liters, (B) 50% tidal with 2.2 liters initial FV, (C) 2.2 liters initial FV with 70% tidal, (D) as in B, with one complete renewal of the initial FV at midsession, (E) 2.2 liters FV with breakpoint modality. Urea, creatinine and phosphate peritoneal clearances, sodium removal and ultrafiltration (UF) were compared using analysis of variance...
2016: Blood Purification
#27
JOURNAL ARTICLE
Paolo Bettica, Stefania Petrini, Valentina D'Oria, Adele D'Amico, Michela Catteruccia, Marika Pane, Serena Sivo, Francesca Magri, Simona Brajkovic, Sonia Messina, Gian Luca Vita, Barbara Gatti, Maurizio Moggio, Pier Lorenzo Puri, Maurizio Rocchetti, Giuseppe De Nicolao, Giuseppe Vita, Giacomo P Comi, Enrico Bertini, Eugenio Mercuri
Duchenne Muscular Dystrophy (DMD) is caused by mutations in the dystrophin gene leading to dystrophin deficiency, muscle fiber degeneration and progressive fibrotic replacement of muscles. Givinostat, a histone deacetylase (HDAC) inhibitor, significantly reduced fibrosis and promoted compensatory muscle regeneration in mdx mice. This study was conducted to evaluate whether the beneficial histological effects of Givinostat could be extended to DMD boys. Twenty ambulant DMD boys aged 7 to <11 years on stable corticosteroid treatment were enrolled in the study and treated for ≥12 months with Givinostat...
October 2016: Neuromuscular Disorders: NMD
#28
MULTICENTER STUDY
Eugenio Mercuri, Giorgia Coratti, Sonia Messina, Valeria Ricotti, Giovanni Baranello, Adele D'Amico, Maria Carmela Pera, Emilio Albamonte, Serena Sivo, Elena Stacy Mazzone, Maria Teresa Arnoldi, Lavinia Fanelli, Roberto De Sanctis, Domenico M Romeo, Gian Luca Vita, Roberta Battini, Enrico Bertini, Francesco Muntoni, Marika Pane
The advent of therapeutic approaches for Duchenne muscular dystrophy (DMD) has highlighted the need to identify reliable outcome measures for young boys with DMD. The aim of this study was to develop a revised version of the North Star Ambulatory Assessment (NSAA) suitable for boys between the age of 3 and 5 years by identifying age appropriate items and revising the scoring system accordingly. Using the scale in 171 controls between the age of 2.9 and 4.8 years, we identified items that were appropriate at different age points...
2016: PloS One
#29
MULTICENTER STUDY
Eugenio Mercuri, Richard Finkel, Jacqueline Montes, Elena S Mazzone, Maria Pia Sormani, Marion Main, Danielle Ramsey, Anna Mayhew, Allan M Glanzman, Sally Dunaway, Rachel Salazar, Amy Pasternak, Janet Quigley, Marika Pane, Maria Carmela Pera, Mariacristina Scoto, Sonia Messina, Maria Sframeli, Gian Luca Vita, Adele D'Amico, Marleen van den Hauwe, Serena Sivo, Nathalie Goemans, Petra Kaufmann, Basil T Darras, Enrico Bertini, Francesco Muntoni, Darryl C De Vivo
The aim of the study was to establish 12-month changes in the Hammersmith Functional motor scale in a large cohort of SMA patients, to identify patterns of disease progression and the effect of different variables. 268 patients were included in this multicentric study. Their age ranged between 2.5 and 55.5 years at baseline, 68 were ambulant and 200 non-ambulant. The baseline scores ranged between 0 and 66 (mean 23.91, SD 20.09). The 12-month change was between -14 and +9 (mean -0.56, SD 2.72). Of the 268 patients, 206 (76...
February 2016: Neuromuscular Disorders: NMD
#30
Marika Pane, Elena Stacy Mazzone, Serena Sivo, Maria Pia Sormani, Sonia Messina, Adele D'Amico, Adelina Carlesi, Gianluca Vita, Lavinia Fanelli, Angela Berardinelli, Yvan Torrente, Valentina Lanzillotta, Emanuela Viggiano, Paola D'Ambrosio, Filippo Cavallaro, Silvia Frosini, Andrea Barp, Serena Bonfiglio, Roberta Scalise, Roberto De Sanctis, Enrica Rolle, Alessandra Graziano, Francesca Magri, Concetta Palermo, Francesca Rossi, Maria Alice Donati, Michele Sacchini, Maria Teresa Arnoldi, Giovanni Baranello, Tiziana Mongini, Antonella Pini, Roberta Battini, Elena Pegoraro, Stefano Previtali, Claudio Bruno, Luisa Politano, Giacomo P Comi, Enrico Bertini, Eugenio Mercuri
No abstract text is available yet for this article.
2015: PloS One
#31
MULTICENTER STUDY
Marika Pane, Lavinia Fanelli, Elena Stacy Mazzone, Giorgia Olivieri, Adele D'Amico, Sonia Messina, Marianna Scutifero, Roberta Battini, Roberta Petillo, Silvia Frosini, Serena Sivo, Gian Luca Vita, Claudio Bruno, Tiziana Mongini, Elena Pegoraro, Roberto De Sanctis, Alice Gardani, Angela Berardinelli, Valentina Lanzillotta, Adelina Carlesi, Emanuela Viggiano, Filippo Cavallaro, Maria Sframeli, Luca Bello, Andrea Barp, Flaviana Bianco, Serena Bonfiglio, Enrica Rolle, Concetta Palermo, Grazia D'Angelo, Antonella Pini, Elena Iotti, Ksenija Gorni, Giovanni Baranello, Enrico Bertini, Luisa Politano, Maria Pia Sormani, Eugenio Mercuri
The aim of this study was to establish the possible effect of glucocorticoid treatment on upper limb function in a cohort of 91 non-ambulant DMD boys and adults of age between 11 and 26 years. All 91 were assessed using the Performance of Upper Limb test. Forty-eight were still on glucocorticoid after loss of ambulation, 25 stopped steroids at the time they lost ambulation and 18 were GC naïve or had steroids while ambulant for less than a year. At baseline the total scores ranged between 0 and 74 (mean 41...
October 2015: Neuromuscular Disorders: NMD
#32
JOURNAL ARTICLE
Elena Mazzone, Jacqueline Montes, Marion Main, Anna Mayhew, Danielle Ramsey, Allan M Glanzman, Sally Dunaway, Rachel Salazar, Amy Pasternak, Janet Quigley, Marika Pane, Maria C Pera, Mariacristina Scoto, Sonia Messina, Maria Sframeli, Adele D'amico, Marleen Van Den Hauwe, Serena Sivo, Nathalie Goemans, Basil T Darras, Petra Kaufmann, Enrico Bertini, Darryl C De Vivo, Francesco Muntoni, Richard Finkel, Eugenio Mercuri
INTRODUCTION: A recent Rasch analysis performed on the Hammersmith Functional Motor Scale-Expanded (HFMSE) in patients with spinal muscular atrophy (SMA) identified issues impacting scale validity, redundant items, and disordered thresholds on some items. METHODS: We modified the HMFSE scoring based on the Rasch analysis and on expert consensus to establish whether the traditional scoring overestimated the number of patients with changes within 2 points from baseline...
September 2015: Muscle & Nerve
#33
JOURNAL ARTICLE
A Togo, D Espadas Maciá, S Blanes Segura, N Sivó Díaz, C Villalba Martínez
INTRODUCTION: Despite the increasing interest in vitamin D functions, new cases of deficiency have been reported in sunny regions where optimal levels are expected. The aim of this study was to analyze 25-hydroxivitamin D levels in children younger than 2 years admitted for acute mild diseases in a tertiary hospital in Valencia and its relationship with factors that can be associated with its deficiency. METHODS: This one year prospective and observational study was conducted on 169 children admitted for acute mild diseases...
March 2016: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
#34
JOURNAL ARTICLE
Marika Pane, Elena Stacy Mazzone, Serena Sivo, Lavinia Fanelli, Roberto De Sanctis, Adele D'Amico, Sonia Messina, Roberta Battini, Flaviana Bianco, Marianna Scutifero, Roberta Petillo, Silvia Frosini, Roberta Scalise, Gian Luca Vita, Claudio Bruno, Marina Pedemonte, Tiziana Mongini, Elena Pegoraro, Francesca Brustia, Alice Gardani, Angela Berardinelli, Valentina Lanzillotta, Emanuela Viggiano, Filippo Cavallaro, Maria Sframeli, Luca Bello, Andrea Barp, Fabio Busato, Serena Bonfiglio, Enrica Rolle, Giulia Colia, Annamaria Bonetti, Concetta Palermo, Alessandra Graziano, Grazia D'Angelo, Antonella Pini, Alice Corlatti, Ksenija Gorni, Giovanni Baranello, Laura Antonaci, Enrico Bertini, Luisa Politano, Eugenio Mercuri
The Performance of Upper Limb (PUL) test was specifically developed for the assessment of upper limbs in Duchenne muscular dystrophy (DMD). The first published data have shown that early signs of involvement can also be found in ambulant DMD boys. The aim of this longitudinal Italian multicentric study was to evaluate the correlation between the 6 Minute Walk Test (6MWT) and the PUL in ambulant DMD boys. Both 6MWT and PUL were administered to 164 ambulant DMD boys of age between 5.0 and 16.17 years (mean 8...
October 7, 2014: PLoS Currents
#35
JOURNAL ARTICLE
Serena Sivo, Elena Mazzone, Laura Antonaci, Roberto De Sanctis, Lavinia Fanelli, Concetta Palermo, Jacqueline Montes, Marika Pane, Eugenio Mercuri
Recent studies have suggested that in non-ambulant patients affected by spinal muscular atrophy the Upper Limb Module can increase the range of activities assessed by the Hammersmith Functional Motor Scale Expanded. The aim of this study was to establish 12-month changes in the Upper Limb Module in a cohort of non-ambulant spinal muscular atrophy patients and their correlation with changes on the Hammersmith Functional Motor Scale Expanded. The Upper Limb Module scores ranged between 0 and 17 (mean 10.23, SD 4...
March 2015: Neuromuscular Disorders: NMD
#36
MULTICENTER STUDY
Roberto De Sanctis, Marika Pane, Serena Sivo, Valeria Ricotti, Giovanni Baranello, Silvia Frosini, Elena Mazzone, Flaviana Bianco, Lavinia Fanelli, Marion Main, Alice Corlatti, Adele D'Amico, Giulia Colia, Roberta Scalise, Concetta Palermo, Chiara Alfonsi, Giovanna Tritto, Domenico M Romeo, Alessandra Graziano, Roberta Battini, Lucia Morandi, Enrico Bertini, Francesco Muntoni, Eugenio Mercuri
The aim of this study was to establish the suitability of the North Star Ambulatory Assessment for use in young boys with Duchenne muscular dystrophy. We studied 147 typically developing and 144 boys affected by Duchenne muscular dystrophy between the ages of 3 and 5 years. More than 85% of the typically developing boys by the age of 4 years had full scores on all the items with total scores ≥33/34. Before the age of 4 years more than 15% of the typically developing boys did not achieve full scores on all the items...
January 2015: Neuromuscular Disorders: NMD
#37
JOURNAL ARTICLE
First on-sky SCAO validation of full LQG control with vibration mitigation on the CANARY pathfinder.
Gaetano Sivo, Caroline Kulcsár, Jean-Marc Conan, Henri-François Raynaud, Eric Gendron, Alastair Basden, Fabrice Vidal, Tim Morris, Serge Meimon, Cyril Petit, Damien Gratadour, Olivier Martin, Zoltan Hubert, Arnaud Sevin, Denis Perret, Fanny Chemla, Gérard Rousset, Nigel Dipper, Gordon Talbot, Eddy Younger, Richard Myers, David Henry, Stephen Todd, David Atkinson, Colin Dickson, Andy Longmore
Adaptive optics provides real time correction of wavefront disturbances on ground based telescopes. Optimizing control and performance is a key issue for ever more demanding instruments on ever larger telescopes affected not only by atmospheric turbulence, but also by vibrations, windshake and tracking errors. Linear Quadratic Gaussian control achieves optimal correction when provided with a temporal model of the disturbance. We present in this paper the first on-sky results of a Kalman filter based LQG control with vibration mitigation on the CANARY instrument at the Nasmyth platform of the 4...
September 22, 2014: Optics Express
#38
JOURNAL ARTICLE
Lorenzo Maggi, Adele D'Amico, Antonella Pini, Serena Sivo, Marika Pane, Giulia Ricci, Liliana Vercelli, Paola D'Ambrosio, Lorena Travaglini, Simone Sala, Greta Brenna, Dimos Kapetis, Marina Scarlato, Elena Pegoraro, Maurizio Ferrari, Antonio Toscano, Sara Benedetti, Pia Bernasconi, Lara Colleoni, Giovanna Lattanzi, Enrico Bertini, Eugenio Mercuri, Gabriele Siciliano, Carmelo Rodolico, Tiziana Mongini, Luisa Politano, Stefano C Previtali, Nicola Carboni, Renato Mantegazza, Lucia Morandi
OBJECTIVES: Our aim was to conduct a comparative study in a large cohort of myopathic patients carrying LMNA gene mutations to evaluate clinical and molecular features associated with different phenotypes. METHODS: We performed a retrospective cohort study of 78 myopathic patients with LMNA mutation and 30 familial cases with LMNA mutation without muscle involvement. We analyzed features characterizing the various forms of LMNA-related myopathy through correlation statistics...
October 28, 2014: Neurology
#39
MULTICENTER STUDY
Marika Pane, Elena Stacy Mazzone, Serena Sivo, Maria Pia Sormani, Sonia Messina, Adele D'Amico, Adelina Carlesi, Gianluca Vita, Lavinia Fanelli, Angela Berardinelli, Yvan Torrente, Valentina Lanzillotta, Emanuela Viggiano, Paola D Ambrosio, Filippo Cavallaro, Silvia Frosini, Andrea Barp, Serena Bonfiglio, Roberta Scalise, Roberto De Sanctis, Enrica Rolle, Alessandra Graziano, Francesca Magri, Concetta Palermo, Francesca Rossi, Maria Alice Donati, Michele Sacchini, Maria Teresa Arnoldi, Giovanni Baranello, Tiziana Mongini, Antonella Pini, Roberta Battini, Elena Pegoraro, Stefano Previtali, Claudio Bruno, Luisa Politano, Giacomo P Comi, Enrico Bertini, Eugenio Mercuri
The 6 minute walk test has been recently chosen as the primary outcome measure in international multicenter clinical trials in Duchenne muscular dystrophy ambulant patients. The aim of the study was to assess the spectrum of changes at 3 years in the individual measures, their correlation with steroid treatment, age and 6 minute walk test values at baseline. Ninety-six patients from 11 centers were assessed at baseline and 12, 24 and 36 months after baseline using the 6 minute walk test and the North Star Ambulatory Assessment...
2014: PloS One
#40
JOURNAL ARTICLE
Pablo R Castello, Iain Hill, Frank Sivo, Lucas Portelli, Frank Barnes, Robert Usselman, Carlos F Martino
This study presents experimental data for the effects of weak radio frequency (RF) magnetic fields on hydrogen peroxide (H2O2) production and cellular growth rates of fibrosarcoma HT1080 cells in vitro. Cells were exposed either to 45 µT static magnetic fields (SMFs)-oriented vertical to the plane of growth or to SMFs combined with weak 5 and 10 MHz RF magnetic fields of 10 µTRMS intensity perpendicular to the static field. Cell numbers were reduced up to 30% on Day 2 for the cells exposed to the combination of SMF and a 10 MHz RF magnetic field compared with the SMF control cells...
December 2014: Bioelectromagnetics
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