In vitro and in vivo characterization of Recifercept, a soluble fibroblast growth factor receptor 3, as treatment for achondroplasia.
PLoS One
; 15(12): e0244368, 2020.
Article
in En
| MEDLINE
| ID: mdl-33370388
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.
Full text:
1
Database:
MEDLINE
Main subject:
Achondroplasia
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Receptor, Fibroblast Growth Factor, Type 3
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Fibroblast Growth Factors
/
Mutation
Type of study:
Prognostic_studies
Limits:
Animals
/
Female
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Humans
/
Male
Language:
En
Year:
2020
Type:
Article