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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 | Artificial neural network optimized by differential evolution for predicting diameters of jet grouted columns显示文摘A novel and effective artificial neural network(ANN) optimized using differential evolution(DE) is first introduced to provide a robust and reliable forecasting of jet grouted column diameters.The proposed computational method adopts the DE algorithm to tackle the difficulties in the training and performance of neural networks and optimize the four quintessential hyper-parameters(i.e.the epoch size,the number of neurons in a hidden layer,the number of hidden layers,and the regularization parameter) that govern the neural network efficacy.This approach is further enhanced by a stochastic gradient optimization algorithm to allow ’expensive’ computation efforts.The ANN-DE is first trained using a prepared jet grouting dataset,then verified and compared with the prevalent machine learning tools,i.e.neural networks and support vector machine(SVM).The results show that,the ANN-DE outperforms the existing methods for predicting the diameter of jet grouting columns since it well balances training efficiency and model performance.Specifically,the ANN-DE achieved root mean square error(RMSE)values of 0.90603 and 0.92813 for the training and testing phases,respectively.The corresponding values were 0.8905 and 0.9006 for the optimized ANN,then,0.87569 and 0.89968 for the optimized SVM,respectively.The proposed paradigm is bound to be useful for solving various geotechnical engineering problems regardless of multi-dimension and nonlinearity. | Pierre Guy Atangana Njock Shui-Long Shen Annan Zhou Giuseppe Modoni | 2021 | Journal of Rock Mechanics and Geotechnical Engineering2021，13，6： | 3 |
| 3 | Longitudinal Evaluation of Vitamin D Status in Healthy Subjects from Southern Italy: Seasonal and Gender Differences 显示文摘 | Camevale V Modoni S Pileri M | 2001 | Osteoporos Int2001，12，12： | 1 |
| 4 | Upgrading equipment and proce- dures for stress path triaxial testing of coarse-grained materials显示文摘 | Flora A Modoni G | 1997 | Geotechnical Testing Journal1997，，4： | 1 |
| 5 | Plasma beta-endorphin response to exercise inpatients with congestive heart failure显示文摘 | PERNA GP MODONI S VALLE G | 1997 | Chest1997，111，1： | 1 |
| 6 | Characterization of the pattern of cognitive impairment in myotonic dystrophy Type 1 显示文摘 | Modoni A Silvestri G Pomponi MG | 2004 | Arch Neurol2004，61，12： | 1 |
| 7 | Theoretical modeling of jet grouting显示文摘 | Modoni G Croce P Mongiovi L | 2006 | Geotechnique2006，56，5： | 1 |
| 8 | Polymorphism of CAG motif of SK3 gene is associated with acute oxaliplatin neurotoxicity显示文摘 | Basso M Modoni A Spada D | 2011 | Cancer Chemother Pharmacol2011，67，5： | 1 |
| 9 | Analysis of foundation reinforced with jet grouting 显示文摘 | Modoni G Bz6wka J | 2012 | Journal of Geotechnical and Geo- environmental Engineering ASCE2012，138，12： | 1 |
| 10 | Analysis of Foundation Re- inforced with Jet Grouting显示文摘 | MODONI G BZOWKA J | 2012 | Journal of Geotechnical and Geoenvironmental Engineering2012，138，12： | 1 |
| 11 | Acute toxicity of cadmium, copper,mercury and zinc to ciliates from activated sludge plants显示文摘 | Modoni P Estebcm G Gorbi G | 1992 | Bull Environ Contam Toxicol1992，49，： | 1 |
| 12 | Spatial analysis of land subsidence induced by groundwater withdrawal显示文摘 | Modoni G Darini G Spacagna R L | 2013 | Engineering Geology2013，167，： | 1 |
| 13 | The diameter of sin- gle, double and triple fluid jet grouting columns:predic- tion method and field trial results 显示文摘 | Flora A Modoni G Liper S | 2013 | Geotechnique2013，63，11： | 1 |
| 14 | Cyclic stress-strain response of compacted gravel显示文摘 | MODONI G KOSEKI J ANH DAN L Q | 2011 | Geotechnique2011，61，6： | 1 |
| 15 | A case of CMT 1B due to Val 102/fs null mutation of the MPZ gene presenting as hyperCKemia显示文摘 | Luigetti M Modoni A Renna R | 2010 | Clin Neurol Neurosurg2010，112，9： | 1 |
| 16 | Evaluation of the use of ultrasonography in the study of liquid gastric emptying显示文摘 | Giacobbe A Conoscitore P Facciorusso D Frusciante V Modoni S | 1989 | Am J Gastroenterol1989，84，5： | 1 |
| 17 | Prevalence of spinocerebellar ataxia type 2 mutation among Italian Parkinsonian patients显示文摘 | Modoni A Contarino MF Bentivoglio AR | 2007 | Mov Disord2007，22，3： | 1 |
| 18 | Theoretical modelling of jet grouting显示文摘 | MODONI G CROCE P MONGIOVI L | 2008 | Geotechnique2008，58，6： | 1 |
| 19 | Polymorphism of CAG motif of SK3 gene is associated with acute oxaliplatin neurotoxicity显示文摘 | Basso M Modoni A Spada D | 2011 | Cancer chemotherapy and pharmacology2011，67，5： | 1 |
| 20 | Theoreticalmodeling offset grouting显示文摘 | MODONI G CROCE P MONGIOVI L | 2008 | Geotechnique2008，58，6： | 1 |
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