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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

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Bone Research 2023年第1期11卷 201-214页

作者： Sharon Russo Federica Scotto di Carlo Antonio Maurizi giorgio fortunato Anna Teti Danilo Licastro Carmine Settembre Tommaso Mello Fernando Gianfrancesco Institute of Genetics and Biophysics“Adriano Buzzati-Traverso” National Research Council of ItalyNaplesItaly Department of Environmental Biological and Pharmaceutical Sciences and Technologies(DiSTABiF)University of Campania Luigi VanvitelliCasertaItaly Department of Biotechnological and Applied Clinical Sciences University of L’AquilaL’AquilaItaly AREA Science Park PadricianoTriesteItaly Telethon Institute of Genetics and Medicine(TIGEM) PozzuoliItaly Gastroenterology Unit Department of Experimental and Clinical Biochemical Sciences“Mario Serio”University of FlorenceFlorenceItaly IRCCS INM Neuromed PozzilliISItaly

Paget’s disease(PDB)is a late-onset bone remodeling disorder with a broad spectrum of symptoms and *** of the most aggressive forms is caused by the P937R mutation in the ZNF687 *** the genetic involvement of ZNF687 in PDB has been extensively studied,the molecular mechanisms underlying this association remain ***,we describe the first Zfp687 knock-in mouse model and demonstrate that the mutation recapitulates the PDB phenotype,resulting in severely altered bone *** 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 ***,osteoblast activity was deregulated,producing disorganized ***,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 ***,we detected osteophytes and intervertebral disc degeneration,outlining for the first time the link between osteoarthritis and PDB in a PDB mouse *** 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 ***,in this mouse model,the mutation was also associated with a high penetrance of hepatocellular ***,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.

关键词： RAW264.7 Paget remodeling

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