A specific requirement for PDGF-C in palate formation and PDGFR-alpha signaling
| Autor: | Hans Boström, Nicole Wong, Andras Nagy, Patrick P.L. Tam, Hao Ding, Mary L. Marazita, L. Leigh Field, Gou Young Koh, Thomas C. Hart, Christer Betsholtz, Meredith P. O'Rourke, Bonny Tsoi, Xiaoli Wu, Injune Kim, Philippe Soriano |
|---|---|
| Rok vydání: | 2004 |
| Předmět: |
medicine.medical_specialty
Platelet-derived growth factor Receptor Platelet-Derived Growth Factor alpha Mesenchyme PDGFRA Spina Bifida Occulta chemistry.chemical_compound Mice Internal medicine Genetics medicine Animals Abnormalities Multiple Mice Knockout Platelet-Derived Growth Factor Lymphokines biology PDGFC Palate Neural tube Gene Expression Regulation Developmental Cell biology Cleft Palate Endocrinology medicine.anatomical_structure Phenotype chemistry Animals Newborn embryonic structures biology.protein Signal transduction Secondary palate Platelet-derived growth factor receptor Signal Transduction |
| Zdroj: | Nature genetics. 36(10) |
| ISSN: | 1061-4036 |
| Popis: | PDGF-C is a member of the platelet-derived growth factor (PDGF) family, which signals through PDGF receptor (PDGFR) alphaalpha and alphabeta dimers. Here we show that Pdgfc(-/-) mice die in the perinatal period owing to feeding and respiratory difficulties associated with a complete cleft of the secondary palate. This phenotype was less severe than that of Pdgfra(-/-) embryos. Pdgfc(-/-) Pdgfa(-/-) embryos developed a cleft face, subepidermal blistering, deficiency of renal cortex mesenchyme, spina bifida and skeletal and vascular defects. Complete loss of function of both ligands, therefore, phenocopied the loss of PDGFR-alpha function, suggesting that both PDGF-A and PDGF-C signal through PDGFR-alpha to regulate the development of craniofacial structures, the neural tube and mesodermal organs. Our results also show that PDGF-C signaling is a new pathway in palatogenesis, different from, and independent of, those previously implicated. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|