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您的检索式：作者名="Danilo RUSSO"
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| 1 | 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 |
| 2 | Functional correlates of skull shape in Chiroptera:feeding and echolocation adaptations显示文摘Morphological,functional,and behavioral adaptations of bats are among the most diverse within mammals.A strong association between bat skull morphology and feeding behavior has been suggested previously.However,morphological variation related to other drivers of adaptation,in particular echolocation,remains understudied.We assessed variation in skull morphology with respect to ecology(diet and emission type)and function(bite force,masticatory muscles and echolocation characteristics)using geometric morphometrics and comparative methods.Our study suggests that variation in skull shape of 10 bat families is the result of adaptations to broad dietary categories and sound emission types(oral or nasal).Skull shape correlates with echolocation parameters only in a subsample of insectivorous species,possibly because they(almost)entirely rely on this sensory system for locating and capturing prey.Insectivores emitting low frequencies are characterized by a ventrally tilted rostrum,a trait not associated with feeding parameters.This result questions the validity of a trade-off between feeding and echolocation function.Our study advances understanding of the relationship between skull morphology and specific features of echolocation and suggests that evolutionary constraints due to echolocation may differ between different groups within the Chiroptera. | Giada GIACOMINI Anthony HERREL Gloriana CHAVERRI Richard P.BROWN Danilo RUSSO Dino SCARAVELLI Carlo MELORO | 2022 | Integrative Zoology2022，17，3： | 1 |
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