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Mice lacking Nf1 in osteochondroprogenitor cells display skeletal dysplasia similar to patients with neurofibromatosis type I

Mutations in NF1 cause neurofibromatosis type I (NF1), a disorder characterized, among other clinical manifestations, by generalized and focal bony lesions. Dystrophic scoliosis and tibial pseudoarthrosis are the most severe skeletal manifestations for which treatment is not satisfactory, emphasizin...

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Detalhes bibliográficos
Main Authors: Wang, Weixi, Nyman, Jeffry S., Ono, Koichiro, Stevenson, David A., Yang, Xiangli, Elefteriou, Florent
Formato: Artigo
Idioma:Inglês
Publicado em: Oxford University Press 2011
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3177652/
https://ncbi.nlm.nih.gov/pubmed/21757497
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1093/hmg/ddr310
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