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| 1 | Processing stoichiometric silicon carbide fibers from polymethylsilane, part I precursor fiber processing显示文摘 | ZHANG Z F SCOTTO C S LAINE R M | 1998 | J Mater Chem1998，8，： | 2 |
| 2 | A mutation in the ZNF687 gene that is responsible for the severe form of Paget's disease of bone causes severely altered bone remodeling and promotes hepatocellular carcinoma onset in a knock-in mouse model显示文摘Paget’s disease(PDB)is a late-onset bone remodeling disorder with a broad spectrum of symptoms and complications.One of the most aggressive forms is caused by the P937R mutation in the ZNF687 gene.Although the genetic involvement of ZNF687 in PDB has been extensively studied,the molecular mechanisms underlying this association remain unclear.Here,we describe the first Zfp687 knock-in mouse model and demonstrate that the mutation recapitulates the PDB phenotype,resulting in severely altered bone remodeling.Through microcomputed tomography analysis,we observed that 8-month-old mutant mice showed a mainly osteolytic phase,with a significant decrease in the trabecular bone volume affecting the femurs and the vertebrae.Conversely,osteoblast activity was deregulated,producing disorganized bone.Notably,this phenotype became pervasive in 16-month-old mice,where osteoblast function overtook bone resorption,as highlighted by the presence of woven bone in histological analyses,consistent with the PDB phenotype.Furthermore,we detected osteophytes and intervertebral disc degeneration,outlining for the first time the link between osteoarthritis and PDB in a PDB mouse model.RNA sequencing of wild-type and Zfp687 knockout RAW264.7 cells identified a set of genes involved in osteoclastogenesis potentially regulated by Zfp687,e.g.,Tspan7,Cpe,Vegfc,and Ggt1,confirming its role in this process.Strikingly,in this mouse model,the mutation was also associated with a high penetrance of hepatocellular carcinomas.Thus,this study established an essential role of Zfp687 in the regulation of bone remodeling,offering the potential to therapeutically treat PDB,and underlines the oncogenic potential of ZNF687. | Sharon Russo Federica Scotto di Carlo Antonio Maurizi Giorgio Fortunato Anna Teti Danilo Licastro Carmine Settembre Tommaso Mello Fernando Gianfrancesco | 2023 | Bone Research2023，11，1： | 2 |
| 3 | Fbxw7o~- and GSK3- mediated degradation of pl00 is a pro-survival mecha- nism in multiple myeloma 显示文摘 | Busino L Millman SE Scotto L | 2012 | Nat Cell Biol2012，14，4： | 1 |
| 4 | Autonomic Healing of Polymer Composites显示文摘 | S R White N R Scottos P H Geubelle | 2001 | Nature2001，409，15： | 1 |
| 5 | Differential expression of peroxisome proliferator-activated receptors:tissue distribution of PPARα,PPARγ in the adult rat显示文摘 | Braissant O Foufelle F Scotto C | 1996 | Endocrinology1996，137，： | 1 |
| 6 | Tn2009, a Tn916-Like elemet containing mef (E) in Streptococcus pneumoniae显示文摘 | Del Grosso M Scotto d'Abusco A Iannelli F | 2004 | Antimicrob Agents Chemother2004，48，6： | 1 |
| 7 | Treatment of mucous and haemorrhoidal prolapse with circular stapler according to longo: personal experience显示文摘 | Scotto Di Carlo E Medolla A Savino F | 2007 | Chir Ital2007，59，3： | 1 |
| 8 | Molecular and biochemical characterization of the recombinant amidase from hyperthermophilic archaeon Sulfolobus solfataricus显示文摘 | Scotto d'Abusco A Ammendola S Scandurra R | 2001 | Extremophiles2001，59，3： | 1 |
| 9 | Differential expression of peroxisome proliferator activated receptor (PPARs): tissue distribution of PPAR-a, -h, and -g in the adult rat 显示文摘 | Braissant O Foufelle F Scotto C | 1996 | Endocrinology1996，137，： | 1 |
| 10 | Citalopram and fluoxetine in major depression: comparison of two clinical trials in a psychiatrist setting and in general practice显示文摘 | Bougerol T Scotto JC Patris M | 1997 | Clin Drug Invest1997，14，： | 1 |
| 11 | Recombinant Ig-like transcript 3-Fc modulates T cell responses via induction of Th anergy and differentiation of CD8^+ T suppressor cells 显示文摘 | Kim-Schulze S Scotto L Vlad G | 2006 | J Immunol2006，176，： | 1 |
| 12 | HDAC inhibitors and decitabine are highly synergistic and associated with unique gene-expression and epigenetic profiles in models of DLBCL显示文摘 | Kalac M Scotto L Marchi E | 2011 | Blood2011，118，： | 1 |
| 13 | Imaging in the evaluation of blunt Pancreatic trauma显示文摘 | Gomez MA Besson M Scotto B | 2004 | J Radiol2004，85，4： | 1 |
| 14 | Differential expression of peroxsome proliferators-activated receptors (PPARs):tissue distribution of PPARα,βandγ in the adult rat显示文摘 | Braissant O Foufelle F Scotto C | 1996 | Endocrinology1996，137，： | 1 |
| 15 | Identification of copy number gain and overexpressed genes on chromosome arm 20q by an integrative genomic approach in cervical cancer:potential role in progression显示文摘 | Scotto L Narayan G Nandula S V | 2008 | Genes Chromosomes Cancer2008，47，9： | 1 |
| 16 | Xeroderma pigmentosum: cutaneous ocular and neurologic abnormalities in 830 published cases 显示文摘 | Kraemer KH Lee MM Scotto J | 1987 | Arch Dermatol1987，123，2： | 1 |
| 17 | The in vitro phosphorylation of P53 by calcium-dependent protein C characterization of a protein-kinase-C-binding site on P53 显示文摘 | Delphin C Huang KP Scotto C | 1997 | FEBS Letter1997，245，： | 1 |
| 18 | Treatment of mucous and haemorrhoidal prolapse with circular stapler according to Longo: personal experience显示文摘 | Scotto di Carlo E Medolla A Savino F | 2007 | Chit Ital2007，59，3： | 1 |
| 19 | Presece of HBV-DNA inspermat ozoa:a possible verticalt ransmission of HBV via thegermline显示文摘 | Hadchouel M Scotto J Huret JL | 1985 | J Med Virol1985，16，1： | 1 |
| 20 | Replication and gene expression of hepatitis B virus in a transgenic mouse that contains the complete viral genome显示文摘 | Farza H Hadchouel M Scotto J Tiollais P Bahinet C Pourcel C | | 0，，： | 1 |
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