Article
Fibroblast growth factor 10 plays a causative role in the tracheal cartilage defects in a mouse model of Apert syndrome.
Pediatric research - 1 Oct 2009
Tiozzo Caterina, De Langhe Stijn, Carraro Gianni, Alam Denise Al, Nagy Andre, Wigfall Clarence, Hajihosseini Mohammad K, Warburton David, Minoo Parviz, Bellusci Saverio
Abstract excerpt
Patients with Apert syndrome (AS) display a wide range of congenital malformations including tracheal stenosis, which is a disease characterized by a uniform cartilaginous sleeve in place of a normally ribbed cartilagenous trachea. We have studied the cellular and molecular basis of this phenotype in a mouse model of AS (Fgfr2c(+/Delta) mice), which shows ectopic expression of Fgfr2b in mesenchymal tissues. Here...
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