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The chaperone activity of 4PBA ameliorates the skeletal phenotype of Chihuahua, a zebrafish model for dominant osteogenesis imperfecta
Classical osteogenesis imperfecta (OI) is a bone disease caused by type I collagen mutations and characterized by bone fragility, frequent fractures in absence of trauma and growth deficiency. No definitive cure is available for OI and to develop novel drug therapies, taking advantage of a repositio...
Autores principales: | Gioia, Roberta, Tonelli, Francesca, Ceppi, Ilaria, Biggiogera, Marco, Leikin, Sergey, Fisher, Shannon, Tenedini, Elena, Yorgan, Timur A., Schinke, Thorsten, Tian, Kun, Schwartz, Jean-Marc, Forte, Fabiana, Wagener, Raimund, Villani, Simona, Rossi, Antonio, Forlino, Antonella |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2017
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5886106/ https://www.ncbi.nlm.nih.gov/pubmed/28475764 http://dx.doi.org/10.1093/hmg/ddx171 |
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