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

PLoS ONE ◽  
2020 ◽  
Vol 15 (12) ◽  
pp. e0244368
Author(s):  
Diogo Gonçalves ◽  
Guylène Rignol ◽  
Pierre Dellugat ◽  
Guido Hartmann ◽  
Stephanie Sarrazy Garcia ◽  
...  
Keyword(s):  
Growth Factor ◽  
Fibroblast Growth Factor ◽  
Bone Growth ◽  
Fibroblast Growth Factor Receptor ◽  
Genetic Disorder ◽  
Growth Factor Receptor ◽  
Model Systems ◽  
Long Bone ◽  
Fibroblast Growth

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.

Crouzon’s syndrome: a new surgical approach

2019 ◽  
Vol 7 (2) ◽  
pp. 18
Author(s):  
Dr. Madhumati Singh ◽  
Dr. Kishore Felix ◽  
Dr. Anjan Kumar Shah
Keyword(s):  
Growth Factor ◽  
Fibroblast Growth Factor ◽  
Bone Growth ◽  
Fibroblast Growth Factor Receptor ◽  
Genetic Disorder ◽  
Growth Factor Receptor ◽  
Fibroblast Growth ◽  
Feeding Problems ◽  
Facial Region ◽  
Crouzon's Syndrome

Crouzon’s syndrome is a rare genetic disorder characterized by distinctive malformations of the skull and facial region, premature cranial suture closure is the most common skull abnormality, optic disc edema and proptosis are among the most common ocular findings. It is a genetic disorder of gene FGFR-2 (Fibroblast Growth Factor Receptor-2) in 95% of cases, and in 5% of cases, FGFR-3 (Fibroblast Growth Factor Receptor-3) mutation occurs.Once a suture becomes fused, growth perpendicular to that suture becomes restricted and the fused bones act as a single body structure. Compensatory growth occurs at the remaining open sutures to allow continued brain growth, resulting in abnormal bone growth and producing facial deformities.In the new born child, some potential problems that may need to be addressed include respiratory difficulties, feeding problems, neurologic complications such as hydrocephalus and the potential risk of developmental delay.We represent a literature review and a rare case of Crouzon’s Syndrome, who wanted facial correction to be done at the age of 24years. We planned two stage surgical procedure, for correction of facial deformity. 

scholarly journals Fibroblast Growth Factor Receptor-1 Is Essential for In Vitro Cardiomyocyte Development

2003 ◽  
Vol 93 (5) ◽  
pp. 414-420 ◽  
Author(s):  
Patrizia Dell’Era ◽  
Roberto Ronca ◽  
Laura Coco ◽  
Stefania Nicoli ◽  
Marco Metra ◽  
...  
Keyword(s):  
Growth Factor ◽  
Fibroblast Growth Factor ◽  
Fibroblast Growth Factor Receptor ◽  
Growth Factor Receptor ◽  
Fibroblast Growth

scholarly journals FGFRL1 Promotes Ovarian Cancer Progression by Crosstalk with Hedgehog Signaling

2018 ◽  
Vol 2018 ◽  
pp. 1-11 ◽  
Author(s):  
Haiyan Tai ◽  
Zhiyong Wu ◽  
Su’an Sun ◽  
Zhigang Zhang ◽  
Congjian Xu
Keyword(s):  
Cell Proliferation ◽  
Growth Factor ◽  
Fibroblast Growth Factor ◽  
Fibroblast Growth Factor Receptor ◽  
Growth Factor Receptor ◽  
Fibroblast Growth ◽  
Hh Signaling ◽  
And Migration

Fibroblast growth factor receptor-like-1 (FGFRL1) has been identified as the fifth fibroblast growth factor receptor. So far, little is known about its biological functions, particularly in cancer development. Here, for the first time, we demonstrated the roles of FGFRL1 in ovarian carcinoma (OC). An array and existing databases were used to investigate the expression profile of FGFRL1 and the relationship between FGFRL1 expression and clinicopathological parameters. FGFRL1 was significantly upregulated in OC patients, and high FGFRL1 expression was correlated with poor prognosis. In vitro cell proliferation, apoptosis and migration assays, and in vivo subcutaneous xenograft tumor models were used to determine the role of FGFRL1. Loss of function of FGFRL1 significantly influenced cell proliferation, apoptosis, and migration of OC cells in vitro and tumor growth in vivo. Chromatin immunoprecipitation PCR analysis and microarray hybridization were performed to uncover the mechanism. FGFRL1 expression could be induced by hypoxia through hypoxia-inducible factor 1α, which directly binds to the promoter elements of FGFRL1. FGFRL1 promoted tumor progression by crosstalk with Hedgehog (Hh) signaling. Taken together, FGFRL1 is a potential predictor and plays an important role in tumor growth and Hh signaling which could serve as potential therapeutic targets for the treatment of OC.

scholarly journals Fibroblast Growth Factor Receptor, a Novel Receptor for Vegetative Insecticidal Protein Vip3Aa

Toxins ◽  
2018 ◽  
Vol 10 (12) ◽  
pp. 546 ◽  
Author(s):  
Kun Jiang ◽  
Xiaoyue Hou ◽  
Lu Han ◽  
Tongtong Tan ◽  
Zhanglei Cao ◽  
...  
Keyword(s):  
Growth Factor ◽  
Fibroblast Growth Factor ◽  
Fibroblast Growth Factor Receptor ◽  
Ex Vivo ◽  
Growth Factor Receptor ◽  
Sf9 Cells ◽  
Fibroblast Growth ◽  
Blotting Experiment ◽  
High Virulence

Vegetative insecticidal proteins (Vips), which are secreted by some Bacillus thuringiensis strains during vegetative growth, exhibit high virulence to many pests. Vip3A proteins have been used commercially both in some bio-insecticides and in transgenic crops; however, compared with insecticidal crystal proteins, the mechanism of action of Vip3A is still unclear. In this work, we indicated that the fibroblast growth factor receptor-like protein (Sf-FGFR) from the membrane of Sf9 cells could bind to Vip3Aa. The interaction between Vip3Aa and Sf-FGFR was confirmed by pull-down assays and dot blotting experiment in vitro. The binding affinity between Vip3Aa and extracellular regions of Sf-FGFR (GST-FGFR-N) was determined by microscale thermophoresis assay (MST). Moreover, Vip3Aa-Flag could be co-immunoprecipitated with Sf-FGFR-V5 ex vivo. Furthermore, knockdown of Sf-FGFR gene in Sf9 cells resulted in reducing the mortality of those cells to Vip3Aa. In summary, our data indicated that Sf-FGFR is a novel receptor for Vip3Aa.

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