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| 1 | Extra-skeletal manifestations in mice affected by Clcn7-dependent autosomal dominant osteopetrosis type 2 clinical and therapeutic implications显示文摘Autosomal dominant osteopetrosis type 2 (ADO2) is a high-density brittle bone disease characterized by bone pain,multiple fractures and skeletal-related events,including nerve compression syndrome and hematological failure.We demonstrated that in mice carrying the heterozygous Clcn7^G213R mutation,whose human mutant homolog CLCN7^G215R affects patients,the clinical impacts of ADO2 extend beyond the skeleton,affecting several other organs.The hallmark of the extra-skeletal alterations is a consistent perivascular fibrosis,associated with high numbers of macrophages and lymphoid infiltrates.Fragmented clinical information in a small cohort of patients confirms extra-skeletal alterations consistent with a systemic disease,in line with the observation that the CLCN7 gene is expressed in many organs.ADO2 mice also show anxiety and depression and their brains exhibit not only perivascular fibrosis but also β-amyloid accumulation and astrogliosis,suggesting the involvement of the nervous system in the pathogenesis of the ADO2 extra-skeletal alterations.Extra-skeletal organs share a similar cellular pathology,confirmed also in vitro in bone marrow mononuclear cells and osteoclasts,characterized by an impairment of the exit pathway of the Clcn7 protein product,ClC7,through the Golgi,with consequent reduced ClC7 expression in late endosomes and lysosomes,associated with high vesicular pH and accumulation of autophagosome markers.Finally,an experimental siRNA therapy,previously proven to counteract the bone phenotype,also improves the extra-skeletal alterations.These results could have important clinical implications,supporting the notion that a systematic evaluation of ADO2 patients for extra-skeletal symptoms could help improve their diagnosis,clinical management,and therapeutic options. | Antonio Maurizi Mattia Capulli Annabel Curle Rajvi Patel Argia Ucci Juliana Alves Cotes Harriet Oxford Shireen R. Lamandé John F. Bateman Nadia Rucci Anna Teti | 2019 | Bone Research2019，7，2： | 3 |
| 2 | Advanced monitoring of machining operations显示文摘 | R. Teti K. Jemielniak G. O’Donnell D. Dornfeld | 2010 | CIRP Annals - Manufacturing Technology2010，，2： | 2 |
| 3 | 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 |
| 4 | Machining of Composite Materials显示文摘 | R. Teti | 2002 | CIRP Annals - Manufacturing Technology2002，，2： | 2 |
| 5 | Optimization of High EPA Structured Phospholipids Synthesis from (u-3 Fatty Acid Enriched Oil and Soy Lecithin显示文摘 | Teti Estiasih Kgs Ahmadi Erliana Ginting Arya Ulil Albab | 2013 | Journal of Food Science and Engineering2013，3，1： | 2 |
| 6 | Sources of parenting stress for depressed and nondepressed mothers of infants显示文摘 | Gelfand DT Teti DM Fox CR | 1992 | Journal of Clinical Child Psychology1992，21，3： | 1 |
| 7 | Parenting Sensitivity, Parental Depression and Child Health: The Mediational Retie of Parental Self- Efficacy显示文摘 | Teti D O'Connell M Reiner C | 1999 | Early Development and Parenting1999，5，4： | 1 |
| 8 | Response to STI571 in chronic myelomonocytic leukemia with platelet derived growth factor beta receptor involvement: a new case report 显示文摘 | Pitini V Arrigo C Teti D | 2003 | Haematologiea2003，88，5： | 1 |
| 9 | Transforming growth factor - beta enhances adhesion of melanoma cells to the endothelitma in vitro显示文摘 | TETI A DE GIORGI A SPINELLA M T | 1997 | Int J Cancer1997，72，6： | 1 |
| 10 | Intelligent computing methods for manufacturing systems显示文摘 | TETI R KUMARA S R T | 1997 | Annals of the CIRP1997，46，2： | 1 |
| 11 | The selective estrogen receptor modulator raloxifene regulates osteoclast and osteoblast activity in vitro显示文摘 | Taranta A Brama M Teti A | 2002 | Bone2002，30，2： | 1 |
| 12 | The selective estrogen recep- tor modulator raloxifene regulates osteoclast and osteoblast activi- ty in vitro 显示文摘 | Taranta A Brama M Teti A | 2002 | Bone2002，30，2： | 1 |
| 13 | Sources of parenting stress for depressed and nondepressed mothers of infants 显示文摘 | Gelfand DM Teti DM Fox CE | 1992 | Journal of Clinical Child Psychology1992，21，3： | 1 |
| 14 | The selective estrogen receptor modulator raloxifene regulates osteoclast and esteoblast activity invitro显示文摘 | Taranta A Brama M Teti A | | 0，，： | 1 |
| 15 | Monocytes from circulating blood fuse in vitro with purified osteoclasts in primary culture显示文摘 | Zambonin Zallone A Teti A Primavera MV | 1984 | J Cell Sci1984，66，： | 1 |
| 16 | Bone metastasis:pathogenesis and therapeutic implications显示文摘 | Clezardin P Teti A | 2007 | Clin Exp Metastasis2007，24，8： | 1 |
| 17 | Immunocy to chemical distribution of extracellular matrix receptors in human osteoclasts:a beta 3 integyin is colocalized with vonculin and talin in the podosomes of oste o clasto ma giant cells显示文摘 | Zambonin -Zallone A Teti A Grano M | 1989 | Exp Cell Res1989，182，2： | 1 |
| 18 | The localization of thyroid hormone receptor mrnas in human bone显示文摘 | Abu EO Homer A Teti A | 2000 | Thyroid2000，10，4： | 1 |
| 19 | Recent advances in engineering design optimization:Challenges and future trends显示文摘 | ROY R HINDUJA S TETI R | 2008 | CIRP Annals Manufacturing Technology2008，57，： | 1 |
| 20 | A muhifeature decision space approach to radar target Identification 显示文摘 | Teti J G Gorman R P Berger W A | 1996 | IEEE Transection on Aerospace And Electronic System1996，32，1： | 1 |
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