| Literature DB >> 33370388 |
Diogo Gonçalves1, Guylène Rignol1, Pierre Dellugat1, Guido Hartmann1,2, Stephanie Sarrazy Garcia3, Jeffrey Stavenhagen4, Luca Santarelli5, Elvire Gouze6, Christian Czech1.
Abstract
Achondroplasia is a rare genetic disorder caused by mutations in the Fibroblast Growth Factor receptor 3 (FGFR3). These mutations lead to aberrant increase of inhibitory signaling in proliferating chondrocytes at the growth plate. Recifercept is a potential treatment for this disease using a decoy approach to sequester FGFR3 ligands subsequently normalizing activation of the mutated FGFR3 receptor. Recifercept binds to FGF isoforms in vitro and in cellular model systems and reduces FGFR3 signaling. In addition, in a transgenic mouse model of achondroplasia, Recifercept restores reduced body weight and long bone growth in these mice. These data suggest that Recifercept treatment could lead to clinical benefits in children treated with this molecule.Entities:
Year: 2020 PMID: 33370388 PMCID: PMC7769458 DOI: 10.1371/journal.pone.0244368
Source DB: PubMed Journal: PLoS One ISSN: 1932-6203 Impact factor: 3.240