Achondroplasia (ACH) is the most frequent form of dwarfism and is caused by gain-of-function mutations in the fibroblast growth factor receptor 3–encoding (FGFR3-encoding) gene. Although potential therapeutic strategies for ACH, which aim to reduce excessive FGFR3 activation, have emerged over many years, the use of tyrosine kinase inhibitor (TKI) to counteract FGFR3 hyperactivity has yet to be evaluated. Here, we have reported that the pan-FGFR TKI, NVP-BGJ398, reduces FGFR3 phosphorylation and corrects the abnormal femoral growth plate and calvaria in organ cultures from embryos of the
Davide Komla-Ebri, Emilie Dambroise, Ina Kramer, Catherine Benoist-Lasselin, Nabil Kaci, Cindy Le Gall, Ludovic Martin, Patricia Busca, Florent Barbault, Diana Graus-Porta, Arnold Munnich, Michaela Kneissel, Federico Di Rocco, Martin Biosse-Duplan, Laurence Legeai-Mallet
Title and authors | Publication | Year |
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Disease-specific complications and multidisciplinary interventions in achondroplasia
H Kitoh, M Matsushita, K Mishima, Y Kamiya, K Sawamura |
Journal of Bone and Mineral Metabolism | 2022 |
Theobroma cacao improves bone growth by modulating defective ciliogenesis in a mouse model of achondroplasia
L Martin, N Kaci, C Benoist-Lasselin, M Mondoloni, S Decaudaveine, V Estibals, M Cornille, L Loisay, J Flipo, B Demuynck, M de la Luz Cádiz-Gurrea, F Barbault, S Fernández-Arroyo, L Schibler, A Segura-Carretero, E Dambroise, L Legeai-Mallet |
Bone Research | 2022 |
Meclozine Attenuates the MARK Pathway in Mammalian Chondrocytes and Ameliorates FGF2-Induced Bone Hyperossification in Larval Zebrafish
G Takemoto, M Matsushita, T Okamoto, T Ito, Y Matsuura, C Takashima, T Chen-Yoshikawa, H Ebi, S Imagama, H Kitoh, K Ohno, Y Hosono |
Frontiers in Cell and Developmental Biology | 2022 |
FGFR3 overactivation in the brain is responsible for memory impairments in Crouzon syndrome mouse model.
Cornille M, Moriceau S, Khonsari RH, Heuzé Y, Loisay L, Boitez V, Morice A, Arnaud E, Collet C, Bensidhoum M, Kaci N, Boddaert N, Paternoster G, Rauschendorfer T, Werner S, Mansour SL, Di Rocco F, Oury F, Legeai-Mallet L |
2022 | |
Infigratinib in children with achondroplasia: the PROPEL and PROPEL 2 studies.
Savarirayan R, De Bergua JM, Arundel P, McDevitt H, Cormier-Daire V, Saraff V, Skae M, Delgado B, Leiva-Gea A, Santos-Simarro F, Salles JP, Nicolino M, Rossi M, Kannu P, Bober MB, Phillips J 3rd, Saal H, Harmatz P, Burren C, Gotway G, Cho T, Muslimova E, Weng R, Rogoff D, Hoover-Fong J, Irving M |
Therapeutic Advances in Musculoskeletal Diseases | 2022 |
Glutathione-Mediated Conjugation of Anticancer Drugs: An Overview of Reaction Mechanisms and Biological Significance for Drug Detoxification and Bioactivation
Potęga A |
Molecules (Basel, Switzerland) | 2022 |
The novel FGFR inhibitor F1-7 induces DNA damage and cell death in colon cells
Liu Y, Zhang L, Chen X, Chen D, Shi X, Song J, Wu J, Huang F, Xia Q, Xiang Y, Zheng X, Cai Y |
British Journal of Cancer | 2022 |
Meclozine ameliorates bone mineralization and growth plate structure in a mouse model of X‑linked hypophosphatemia
Kamiya Y, Matsushita M, Mishima K, Ohkawara B, Michigami T, Imagama S, Ohno K, Kitoh H |
Experimental and therapeutic medicine | 2022 |
New developments in the biology of fibroblast growth factors
Ornitz DM, Itoh N |
2022 |