| Fibroblast growth factor 10 plays a causative role in the tracheal cartilage defects in a mouse model of Apert syndrome. | |
| | |
MedLine Citation:
|
PMID: 19581825 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Patients with Apert syndrome (AS) display a wide range of congenital malformations including tracheal stenosis, which is a disease characterized by a uniform cartilaginous sleeve in place of a normally ribbed cartilagenous trachea. We have studied the cellular and molecular basis of this phenotype in a mouse model of AS (Fgfr2c(+/Delta) mice), which shows ectopic expression of Fgfr2b in mesenchymal tissues. Here we report that tracheal stenosis is associated with increased proliferation of mesenchymal cells, where the expression of Fgf10 and its upstream regulators Tbx4 and Tbx5 are abnormally elevated. We show that Fgf10 has a critical inductive role in tracheal stenosis, as genetic knockdown of Fgf10 in Fgfr2c(+/Delta) mice rescues this phenotype. These novel findings demonstrate a regulatory role for Fgf10 in tracheal development and shed more light on the underlying cause of tracheal defects in AS. |
| | |
Authors:
|
Caterina Tiozzo; Stijn De Langhe; Gianni Carraro; Denise Al Alam; Andre Nagy; Clarence Wigfall; Mohammad K Hajihosseini; David Warburton; Parviz Minoo; Saverio Bellusci |
Related Documents
:
|
8352155 - Kallmann syndrome: mr findings. 9498385 - Anorectal malformations and down's syndrome. 11807865 - Craniosynostosis, ectopia lentis, and congenital heart defects: further delineation of ... 9799305 - Otodental syndrome, oculo-facio-cardio-dental (ofcd) syndrome, and lobodontia: dental d... 7196955 - Monozygotic twins discordant for rubinstein-taybi syndrome. 21132565 - Hughes syndrome (the antiphospholipid syndrome): a disease of our time. |
Publication Detail:
|
Type: Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't |
Journal Detail:
|
Title: Pediatric research Volume: 66 ISSN: 1530-0447 ISO Abbreviation: Pediatr. Res. Publication Date: 2009 Oct |
Date Detail:
|
Created Date: 2009-12-17 Completed Date: 2010-02-05 Revised Date: - |
Medline Journal Info:
|
Nlm Unique ID: 0100714 Medline TA: Pediatr Res Country: United States |
Other Details:
|
Languages: eng Pagination: 386-90 Citation Subset: IM |
Affiliation:
|
Department of Pediatrics, Women's and Children's Hospital, University of Southern California Keck School of Medicine, Los Angeles, California 90033, USA. |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Acrocephalosyndactylia
/
metabolism,
pathology* Animals Body Patterning Cartilage / abnormalities* Embryo, Mammalian / anatomy & histology, physiology Fibroblast Growth Factor 10 / genetics, metabolism* Humans Mice Mice, Transgenic Phenotype Receptor, Fibroblast Growth Factor, Type 2 / genetics, metabolism Signal Transduction / physiology T-Box Domain Proteins / genetics, metabolism Trachea / anatomy & histology* |
| Grant Support | |
ID/Acronym/Agency:
|
1R01HL086322/HL/NHLBI NIH HHS |
| Chemical | |
Reg. No./Substance:
|
0/Fibroblast Growth Factor 10; 0/T-Box Domain Proteins; 0/Tbx4 protein, mouse; EC 2.7.10.1/Receptor, Fibroblast Growth Factor, Type 2 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Breast-feeding is associated with a reduced frequency of acute otitis media and high serum antibody ...
Next Document: Characterization and culture of fetal rhesus monkey renal cortical cells.