Literature DB >> 33370388

In vitro and in vivo characterization of Recifercept, a soluble fibroblast growth factor receptor 3, as treatment for achondroplasia.

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


  27 in total

1.  Up-regulation of the chondrogenic Sox9 gene by fibroblast growth factors is mediated by the mitogen-activated protein kinase pathway.

Authors:  S Murakami; M Kan; W L McKeehan; B de Crombrugghe
Journal:  Proc Natl Acad Sci U S A       Date:  2000-02-01       Impact factor: 11.205

2.  Skeletal dysplasia and defective chondrocyte differentiation by targeted overexpression of fibroblast growth factor 9 in transgenic mice.

Authors:  S Garofalo; M Kliger-Spatz; J L Cooke; O Wolstin; G P Lunstrum; S M Moshkovitz; W A Horton; A Yayon
Journal:  J Bone Miner Res       Date:  1999-11       Impact factor: 6.741

Review 3.  FGF signaling in the developing endochondral skeleton.

Authors:  David M Ornitz
Journal:  Cytokine Growth Factor Rev       Date:  2005-04-01       Impact factor: 7.638

Review 4.  Cell responses to FGFR3 signalling: growth, differentiation and apoptosis.

Authors:  Corine G M L'Hôte; Margaret A Knowles
Journal:  Exp Cell Res       Date:  2004-12-16       Impact factor: 3.905

5.  FGF2 inhibits proliferation and alters the cartilage-like phenotype of RCS cells.

Authors:  Pavel Krejci; Vitezslav Bryja; Jiri Pachernik; Ales Hampl; Robert Pogue; Pertchoui Mekikian; William R Wilcox
Journal:  Exp Cell Res       Date:  2004-07-01       Impact factor: 3.905

6.  Sustained phosphorylation of mutated FGFR3 is a crucial feature of genetic dwarfism and induces apoptosis in the ATDC5 chondrogenic cell line via PLCgamma-activated STAT1.

Authors:  Daisuke Harada; Yoshitaka Yamanaka; Koso Ueda; Riko Nishimura; Tsuneo Morishima; Yoshiki Seino; Hiroyuki Tanaka
Journal:  Bone       Date:  2007-02-09       Impact factor: 4.398

7.  Achondroplasia is defined by recurrent G380R mutations of FGFR3.

Authors:  G A Bellus; T W Hefferon; R I Ortiz de Luna; J T Hecht; W A Horton; M Machado; I Kaitila; I McIntosh; C A Francomano
Journal:  Am J Hum Genet       Date:  1995-02       Impact factor: 11.025

Review 8.  Fibroblast growth factor signaling in skeletal development and disease.

Authors:  David M Ornitz; Pierre J Marie
Journal:  Genes Dev       Date:  2015-07-15       Impact factor: 11.361

9.  Final adult height in long-term growth hormone-treated achondroplasia patients.

Authors:  Daisuke Harada; Noriyuki Namba; Yuki Hanioka; Kaoru Ueyama; Natsuko Sakamoto; Yukako Nakano; Masafumi Izui; Yuiko Nagamatsu; Hiroko Kashiwagi; Miho Yamamuro; Yoshihito Ishiura; Ayako Ogitani; Yoshiki Seino
Journal:  Eur J Pediatr       Date:  2017-05-13       Impact factor: 3.183

10.  Repression of hedgehog signaling and BMP4 expression in growth plate cartilage by fibroblast growth factor receptor 3.

Authors:  M C Naski; J S Colvin; J D Coffin; D M Ornitz
Journal:  Development       Date:  1998-12       Impact factor: 6.868

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  4 in total

Review 1.  Disease-specific complications and multidisciplinary interventions in achondroplasia.

Authors:  Hiroshi Kitoh; Masaki Matsushita; Kenichi Mishima; Yasunari Kamiya; Kenta Sawamura
Journal:  J Bone Miner Metab       Date:  2022-01-14       Impact factor: 2.626

2.  Longitudinal Imaging of the Skull Base Synchondroses Demonstrate Prevention of a Premature Ossification After Recifercept Treatment in Mouse Model of Achondroplasia.

Authors:  Guylene Rignol; Stephanie Garcia; Florence Authier; Kaamula Smith; Lionel Tosello; Raphael Marsault; Pierre Dellugat; Diogo Goncalves; Marlene Brouillard; Jeffrey Stavenhagen; Luca Santarelli; Christian Czech; Elvire Gouze
Journal:  JBMR Plus       Date:  2021-11-09

Review 3.  Cranial Base Synchondrosis: Chondrocytes at the Hub.

Authors:  Shawn A Hallett; Wanida Ono; Renny T Franceschi; Noriaki Ono
Journal:  Int J Mol Sci       Date:  2022-07-15       Impact factor: 6.208

Review 4.  Advantages and Disadvantages of Different Treatment Methods in Achondroplasia: A Review.

Authors:  Wiktoria Wrobel; Emilia Pach; Iwona Ben-Skowronek
Journal:  Int J Mol Sci       Date:  2021-05-25       Impact factor: 5.923

  4 in total

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