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A specific requirement for PDGF-C in palate formation and PDGFR-alpha signaling.

Ding, Hao; Wu, Xiaoli; Ehrencrona, Hans LU ; Kim, Injune; Wong, Nicole; Tsoi, Bonny; O'Rourke, Meredith; Koh, Gou Young; Soriano, Philippe and Betsholtz, Christer, et al. (2004) In Nature Genetics 36(10). p.1111-1116
Abstract
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... (More)
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. (Less)
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Phenotype, Palate, Knockout, Mice, Lymphokines, Developmental, Gene Expression Regulation, Cleft Palate, Newborn, Multiple, Abnormalities, Animals, Platelet-Derived Growth Factor, Receptor, Platelet-Derived Growth Factor alpha, Signal Transduction, Spina Bifida Occulta
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Nature Genetics
volume
36
issue
10
pages
1111 - 1116
publisher
Nature Publishing Group
ISSN
1546-1718
language
English
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no
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d09b54a0-4c29-47b3-b30b-ba52f6cfc20d (old id 4021852)
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http://www.ncbi.nlm.nih.gov/pubmed/15361870
http://dx.doi.org/10.1038/ng1415
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2013-10-21 10:53:43
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2016-06-29 09:09:03
@article{d09b54a0-4c29-47b3-b30b-ba52f6cfc20d,
  abstract     = {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.},
  author       = {Ding, Hao and Wu, Xiaoli and Ehrencrona, Hans and Kim, Injune and Wong, Nicole and Tsoi, Bonny and O'Rourke, Meredith and Koh, Gou Young and Soriano, Philippe and Betsholtz, Christer and Hart, Thomas C. and Marazita, Mary L. and Field, L. L. and Tam, Patrick P. L. and Nagy, Andras},
  issn         = {1546-1718},
  keyword      = {Phenotype,Palate,Knockout,Mice,Lymphokines,Developmental,Gene Expression Regulation,Cleft Palate,Newborn,Multiple,Abnormalities,Animals,Platelet-Derived Growth Factor,Receptor,Platelet-Derived Growth Factor alpha,Signal Transduction,Spina Bifida Occulta},
  language     = {eng},
  number       = {10},
  pages        = {1111--1116},
  publisher    = {Nature Publishing Group},
  series       = {Nature Genetics},
  title        = {A specific requirement for PDGF-C in palate formation and PDGFR-alpha signaling.},
  volume       = {36},
  year         = {2004},
}