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| 1 | Resveratrol corrects aberrant splicing of RYR1 pre-mRNA and Ca2+ signal in myotonic dystrophy type 1 myotubes显示文摘Myotonic dystrophy type 1(DM1) is a spliceopathy related to the mis-splicing of several genes caused by sequestration of nuclear transcriptional RNA-binding factors from non-coding CUG repeats of DMPK pre-mRNAs. Dysregulation of ryanodine receptor 1(RYR1), sarcoplasmatic/endoplasmatic Ca^2+-ATPase(SERCA) and α1 S subunit of voltage-gated Ca^2+ channels(Cav1.1) is related to Ca^2+ homeostasis and excitation-contraction coupling impairment. Though no pharmacological treatment for DM1 exists, aberrant splicing correction represents one major therapeutic target for this disease. Resveratrol(RES, 3,5,4′-trihydroxy-trans-stilbene) is a promising pharmacological tools for DM1 treatment for its ability to directly bind the DNA and RNA influencing gene expression and alternative splicing. Herein, we analyzed the therapeutic effects of RES in DM1 myotubes in a pilot study including cultured myotubes from two DM1 patients and two healthy controls. Our results indicated that RES treatment corrected the aberrant splicing of RYR1, and this event appeared associated with restoring of depolarization-induced Ca^2+ release from RYR1 dependent on the electro-mechanical coupling between RYR1 and Cav1.1. Interestingly, immunoblotting studies showed that RES treatment was associated with a reduction in the levels of CUGBP Elav-like family member 1, while RYR1, Cav1.1 and SERCA1 protein levels were unchanged. Finally, RES treatment did not induce any major changes either in the amount of ribonuclear foci or sequestration of muscleblind-like splicing regulator 1. Overall, the results of this pilot study would support RES as an attractive compound for future clinical trials in DM1. Ethical approval was obtained from the Ethical Committee of IRCCS Fondazione Policlinico Universitario A. Gemelli, Rome, Italy(rs9879/14) on May 20, 2014. | Massimo Santoro Roberto Piacentini Alessia Perna Eugenia Pisano Anna Severino Anna Modoni Claudio Grassi Gabriella Silvestri | 2020 | Neural Regeneration Research2020，15，9： | 3 |
| 2 | Current evidence and future research needs for FeNO measurement in respiratory diseases显示文摘 | Leif Bjermer Kjell Alving Zuzana Diamant Helgo Magnussen Ian Pavord Giorgio Piacentini David Price Nicolas Roche Joaquin Sastre Mike Thomas Omar Usmani | 2014 | Respiratory Medicine2014，，： | 2 |
| 3 | Low-dose interleukin-2 administered pre-operatively topatients with gastric cancer activates peripheral and perit-umoral lymphocytes but does not affect prognosis显示文摘 | Giovanni Carlo Cesana Fabrizio Romano Gaia Piacentini | 2007 | Annals Surgical Oncology2007，14，4： | 1 |
| 4 | On the influence of tool path in friction stir spot welding of aluminum alloys显示文摘 | Buffa G Fratini L Piacentini M | 2008 | Journal of Materials Processing Technology2008，208，13： | 1 |
| 5 | Investigation on the effects of a mechanical shear-stress modification method during cross-flow membrane emulsification显示文摘 | Andras Koris Emma Piacentini Gyula Vatai Erika Bekassy-Molnar Enrico Drioli Lidietta Giorno | 2011 | Journal of Membrane Science2011，，1： | 1 |
| 6 | Reduction of subjective distress in CBT for childhood OCD: nature of change, predictors, and relation to treatment outeom显示文摘 | Kireanski K Wu M Piacentini J | 2014 | J Anxiety Disord2014，28，2： | 1 |
| 7 | Endothelin-1 Stimulates Cardiac Fibroblast Proliferation Through Activation of Protein Kinase C显示文摘 | Lucia Piacentini Mary Gray Norman Y Honbo Jamila Chentoufi Marina Bergman Joel S Karliner | 2000 | Journal of Molecular and Cellular Cardiology2000，，4： | 1 |
| 8 | Achievements and unmet needs in the management of advanced ovarian cancer显示文摘 | Guarneri V Piacentini F Barbieri E | 2010 | GynecolOncol2010，117，2： | 1 |
| 9 | Isolation of cancer stem cells from three human glioblastoma cell lines: characterization of two selected clones显示文摘 | Iacopino F Angelucci C Piacentini R | 2014 | PloS One2014，9，10： | 1 |
| 10 | Platinum doublets as first-line treatment for elderly patients with advanced non- small cell lung cancer 显示文摘 | Piacentini P Greco F Mercanti A | 2013 | Tumori2013，99，6： | 1 |
| 11 | Vitamin D Serum Levels and Markers of Asthma Control in Italian Children显示文摘 | Iolanda Chinellato Michele Piazza Marco Sandri Diego Peroni Giorgio Piacentini Attilio L. Boner | 2011 | The Journal of Pediatrics2011，，3： | 1 |
| 12 | Exhaled nitric oxide and sputum eosinophil markers of inflammation in asthmatic children显示文摘 | Piacentini GL Bodini A Costella S | 1999 | Eur Respir J1999，13，： | 1 |
| 13 | Influence of brain catecholamines on the development of fatigue in ex- ercising rats in the heat 显示文摘 | Hasegawa H Piacentini MF Sarre S | 2008 | J Physiol2008，586，1： | 1 |
| 14 | Heterogenous patterns of sensory dysfunction in postherpetic neuralgia suggest multiple pathophysiologic mechanisms 显示文摘 | Pappagallo M Oaklander AL Quatrano Piacentinial | 2000 | Anesthesiology2000，92，3： | 1 |
| 15 | GSTO1 E155del polymorphism associated with increased risk for late-onset Alzheimer's disease: association hypothesis for an uncommon genetic variant 显示文摘 | Piacentini S Polimanti R Squitti R | 2012 | Neurosci Lett2012，506，： | 1 |
| 16 | Quantitative PCR analysis of two molluscan metallothionein genes unveils differential expression and regulation 显示文摘 | Dondero F Piacentini L Banni M | 2005 | Gene2005，345，2： | 1 |
| 17 | Transglutaminase 2: an enigmatic enzyme with diverse functions显示文摘 | Fesus L Piacentini M | 2002 | Trends Biochem Sei2002，27，10： | 1 |
| 18 | An extension of metric temporal planning with application to AC voltage control显示文摘 | Piacentini C Alimisis V Fox M | 2015 | Artificial Intelligence2015，229，12： | 1 |
| 19 | Brain microdialysis in exercise research显示文摘 | Meeusen R Piacentini MF De Meirleir K | 2001 | Sports Med2001，31，14： | 1 |
| 20 | Conjoined lumbosacral nerve roots: observations on three cases and review of the literature 显示文摘 | Artico M Carloia S Piacentini M | 2006 | Neurocirugia2006，17，： | 1 |
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