Click here to close
Hello! We notice that you are using Internet Explorer, which is not supported by Xenbase and may cause the site to display incorrectly.
We suggest using a current version of Chrome,
FireFox, or Safari.
???displayArticle.abstract??? FGFRL1 (fibroblast growth factor receptor like 1) is the most recently discovered member of the FGFR family. It contains three extracellular Ig-like domains similar to the classical FGFRs, but it lacks the protein tyrosine kinase domain and instead contains a short intracellular tail with a peculiar histidine-rich motif. The gene for FGFRL1 is found in all metazoans from sea anemone to mammals. FGFRL1 binds to FGF ligands and heparin with high affinity. It exerts a negative effect on cell proliferation, but a positive effect on cell differentiation. Mice with a targeted deletion of the Fgfrl1 gene die perinatally due to alterations in their diaphragm. These mice also show bilateral kidney agenesis, suggesting an essential role for Fgfrl1 in kidney development. A human patient with a frameshift mutation exhibits craniosynostosis, arguing for an additional role of FGFRL1 during bone formation. FGFRL1 contributes to the complexity of the FGF signaling system.
Amaya,
FGF signalling in the early specification of mesoderm in Xenopus.
1993, Pubmed,
Xenbase
Amaya,
FGF signalling in the early specification of mesoderm in Xenopus.
1993,
Pubmed
,
Xenbase Baertschi,
Mice with a targeted disruption of the Fgfrl1 gene die at birth due to alterations in the diaphragm.
2007,
Pubmed Battaglia,
Wolf-Hirschhorn (4p-) syndrome.
2001,
Pubmed Beenken,
The FGF family: biology, pathophysiology and therapy.
2009,
Pubmed Bertrand,
FGFRL1 is a neglected putative actor of the FGF signalling pathway present in all major metazoan phyla.
2009,
Pubmed Beyeler,
Fgfrl1, a fibroblast growth factor receptor-like gene, is found in the cephalochordate Branchiostoma floridae but not in the urochordate Ciona intestinalis.
2006,
Pubmed Casaccia,
Distal 4p microdeletion in a case of Wolf-Hirschhorn syndrome with congenital diaphragmatic hernia.
2006,
Pubmed Catela,
Multiple congenital malformations of Wolf-Hirschhorn syndrome are recapitulated in Fgfrl1 null mice.
2009,
Pubmed Cebrià,
FGFR-related gene nou-darake restricts brain tissues to the head region of planarians.
2002,
Pubmed
,
Xenbase Chen,
Cell-cell fusion.
2007,
Pubmed Crocker,
Siglecs as positive and negative regulators of the immune system.
2008,
Pubmed Daëron,
Immunoreceptor tyrosine-based inhibition motifs: a quest in the past and future.
2008,
Pubmed Davis,
Fc receptor-like molecules.
2007,
Pubmed Engbers,
Wolf-Hirschhorn syndrome facial dysmorphic features in a patient with a terminal 4p16.3 deletion telomeric to the WHSCR and WHSCR 2 regions.
2009,
Pubmed Eswarakumar,
Cellular signaling by fibroblast growth factor receptors.
2005,
Pubmed Falls,
Genomic imprinting: implications for human disease.
1999,
Pubmed Gerber,
The murine Fgfrl1 receptor is essential for the development of the metanephric kidney.
2009,
Pubmed Greber,
Silencing of core transcription factors in human EC cells highlights the importance of autocrine FGF signaling for self-renewal.
2007,
Pubmed Gubler,
Renin-angiotensin system in kidney development: renal tubular dysgenesis.
2010,
Pubmed Hall,
An essential role for zebrafish Fgfrl1 during gill cartilage development.
2006,
Pubmed Hanaoka,
Zebrafish gcmb is required for pharyngeal cartilage formation.
2004,
Pubmed Hayashi,
Expression patterns of Xenopus FGF receptor-like 1/nou-darake in early Xenopus development resemble those of planarian nou-darake and Xenopus FGF8.
2004,
Pubmed
,
Xenbase Hogan,
Zebrafish gcm2 is required for gill filament budding from pharyngeal ectoderm.
2004,
Pubmed Holder,
Genetic factors in congenital diaphragmatic hernia.
2007,
Pubmed Huang,
Hypoxia-inducible mir-210 regulates normoxic gene expression involved in tumor initiation.
2009,
Pubmed Ibrahimi,
Biochemical analysis of pathogenic ligand-dependent FGFR2 mutations suggests distinct pathophysiological mechanisms for craniofacial and limb abnormalities.
2004,
Pubmed Itoh,
Evolution of the Fgf and Fgfr gene families.
2004,
Pubmed Jackson,
8p11 myeloproliferative syndrome: a review.
2010,
Pubmed Kan,
An essential heparin-binding domain in the fibroblast growth factor receptor kinase.
1993,
Pubmed Kim,
A novel fibroblast growth factor receptor-5 preferentially expressed in the pancreas(1).
2001,
Pubmed Knowles,
Role of FGFR3 in urothelial cell carcinoma: biomarker and potential therapeutic target.
2007,
Pubmed Kurosu,
The Klotho gene family as a regulator of endocrine fibroblast growth factors.
2009,
Pubmed Lazarus,
Fibroblast growth factor expression in the postnatal growth plate.
2007,
Pubmed Long,
Negative signaling by inhibitory receptors: the NK cell paradigm.
2008,
Pubmed Loo,
Binding of heparin/heparan sulfate to fibroblast growth factor receptor 4.
2001,
Pubmed LopezJimenez,
Examination of FGFRL1 as a candidate gene for diaphragmatic defects at chromosome 4p16.3 shows that Fgfrl1 null mice have reduced expression of Tpm3, sarcomere genes and Lrtm1 in the diaphragm.
2010,
Pubmed Luedi,
Computational and experimental identification of novel human imprinted genes.
2007,
Pubmed Mantovani,
Decoy receptors: a strategy to regulate inflammatory cytokines and chemokines.
2001,
Pubmed Mohammadi,
A protein canyon in the FGF-FGF receptor dimer selects from an à la carte menu of heparan sulfate motifs.
2005,
Pubmed Mohammadi,
Structural basis for fibroblast growth factor receptor activation.
2005,
Pubmed Ogawa,
Planarian fibroblast growth factor receptor homologs expressed in stem cells and cephalic ganglions.
2002,
Pubmed Oren-Suissa,
Evolution of programmed cell fusion: common mechanisms and distinct functions.
2010,
Pubmed Pinheiro da Silva,
Inhibitory ITAMs: a matter of life and death.
2008,
Pubmed Plotnikov,
Crystal structures of two FGF-FGFR complexes reveal the determinants of ligand-receptor specificity.
2000,
Pubmed Plotnikov,
Structural basis for FGF receptor dimerization and activation.
1999,
Pubmed Powell,
Fibroblast growth factor receptors 1 and 2 interact differently with heparin/heparan sulfate. Implications for dynamic assembly of a ternary signaling complex.
2002,
Pubmed Putnam,
Sea anemone genome reveals ancestral eumetazoan gene repertoire and genomic organization.
2007,
Pubmed Rieckmann,
The cell surface receptor FGFRL1 forms constitutive dimers that promote cell adhesion.
2008,
Pubmed Rieckmann,
Characterization of the first FGFRL1 mutation identified in a craniosynostosis patient.
2009,
Pubmed Saxén,
Early organogenesis of the kidney.
1987,
Pubmed Schild,
Aberrant expression of FGFRL1, a novel FGF receptor, in ovarian tumors.
2005,
Pubmed Sleeman,
Identification of a new fibroblast growth factor receptor, FGFR5.
2001,
Pubmed Steinberg,
The FGFRL1 receptor is shed from cell membranes, binds fibroblast growth factors (FGFs), and antagonizes FGF signaling in Xenopus embryos.
2010,
Pubmed
,
Xenbase Steinberg,
Rapid fusion and syncytium formation of heterologous cells upon expression of the FGFRL1 receptor.
2010,
Pubmed Trueb,
Expression of FGFRL1, a novel fibroblast growth factor receptor, during embryonic development.
2006,
Pubmed Trueb,
Fish possess multiple copies of fgfrl1, the gene for a novel FGF receptor.
2005,
Pubmed Trueb,
Characterization of FGFRL1, a novel fibroblast growth factor (FGF) receptor preferentially expressed in skeletal tissues.
2003,
Pubmed Urakawa,
Klotho converts canonical FGF receptor into a specific receptor for FGF23.
2006,
Pubmed Wiedemann,
Characterization of a novel protein (FGFRL1) from human cartilage related to FGF receptors.
2000,
Pubmed Wiedemann,
The mouse Fgfrl1 gene coding for a novel FGF receptor-like protein.
2001,
Pubmed Wilkie,
Bad bones, absent smell, selfish testes: the pleiotropic consequences of human FGF receptor mutations.
2005,
Pubmed Zhuang,
Comparison of the receptor FGFRL1 from sea urchins and humans illustrates evolution of a zinc binding motif in the intracellular domain.
2009,
Pubmed Zhuang,
Genome-wide comparison of FGFRL1 with structurally related surface receptors.
2010,
Pubmed
