Article
The chaperone activity of 4PBA ameliorates the skeletal phenotype of Chihuahua, a zebrafish model for dominant osteogenesis imperfecta.
Human molecular genetics - 1 Aug 2017
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
Abstract excerpt
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 repositioning strategy, the small teleost zebrafish (Danio rerio) is a particularly appealing model. Its small size, high...
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