Defective lysosomal targeting of activated fibroblast growth factor receptor 3 in achondroplasia [PDF]
Mutations of fibroblast growth factor receptor 3 (FGFR3) are responsible for achondroplasia (ACH) and related dwarfing conditions in humans. The pathogenesis involves constitutive activation of FGFR3, which inhibits proliferation and differentiation of growth plate chondrocytes.
Jay Y. Cho +6 more
openalex +3 more sources
A novel S269C mutation in fibroblast growth factor receptor 3 in a Japanese child with hypochondroplasia. [PDF]
Takahashi I +6 more
europepmc +3 more sources
Achondroplasia is the most common form of short‐limb dwarfism. In this disorder, endochondral ossification is impaired due to gain‐of‐function mutation in the Fibroblast Growth Factor Receptor 3 (FGFR3) gene.
Guylene Rignol +12 more
doaj +1 more source
Fibroblast growth factor receptor 3 deficiency does not impair the osteoanabolic action of parathyroid hormone on mice [PDF]
Yangli Xie +5 more
doaj +2 more sources
Introduction Advances in the treatment of biliary tract cancer have been made possible through gains in genomic and epigenetic tumor understanding. The use of fibroblast growth factor receptor inhibitor has enabled significant clinical improvement in a ...
Andreas Edwin Juarso +2 more
doaj +1 more source
Cysteine-rich Fibroblast Growth Factor Receptor Alters Secretion and Intracellular Routing of Fibroblast Growth Factor 3 [PDF]
Expression of the cysteine-rich fibroblast growth factor (FGF) receptor (CFR) in COS-1 cells strongly inhibits the secretion of co-expressed FGF3. By using a column retention assay and affinity chromatography, we demonstrate that at physiological salt concentrations FGF3 binds with strong affinity to CFR in vivo and in vitro.
R, Köhl +4 more
openaire +2 more sources
VEGFR1-tyrosine kinase signaling in pulmonary fibrosis
Vascular endothelial growth factor (VEGF) is not only an important factor for angiogenesis but also lung development and homeostasis. VEGF-A binds three tyrosine kinase (TK) receptors VEGFR1–3.
Hideki Amano +4 more
doaj +1 more source
Fibroblast Growth Factor Receptor 3 Gene: Regulation by Serum Response Factor [PDF]
We have previously identified a cis-acting sequence in the proximal promoter of the fibroblast growth factor receptor 3 (FGFR3) gene that strongly activates transcription in chondrocytic cells. Here we report that the transcriptional activity of this sequence (FRE3) requires serum response factor and its cognate recognition motif, serum response ...
Martina I, Reinhold +2 more
openaire +2 more sources
PPAR? Downregulation by TGF in Fibroblast and Impaired Expression and Function in Systemic Sclerosis: A Novel Mechanism for Progressive Fibrogenesis [PDF]
The nuclear orphan receptor peroxisome proliferator-activated receptor-gamma (PPAR-γ) is expressed in multiple cell types in addition to adipocytes. Upon its activation by natural ligands such as fatty acids and eicosanoids, or by synthetic agonists such
A Galli +75 more
core +13 more sources
Effect of periosteal resection on longitudinal bone growth in a mouse model of achondroplasia
Achondroplasia (ACH) is the most common form of short-limbed skeletal dysplasia. Patients with ACH sometimes undergo lower limb lengthening to get functional and psychological achievements.
Shinya Kaneko +5 more
doaj +1 more source

