An activating mutation in the PDGF receptor alpha results in embryonic lethality caused by malformation of the vascular system

  • Platelet-derived growth factors (PDGF) and their receptors control cell proliferation, survival, and migration. To test the influence of an oncogenic mutation to embryonic development, a transgenic mouse line expressing PDGFRalpha (D842V) was established and analyzed. Most of the embryos die on embryonic day 12.5 due to massive hemorrhages in the trunk. In mesenchymal cells of mutant animals, proliferation is decreased while apoptosis is increased. Further analyses reveal that the aortic blood vessels are enlarged showing a reduced numbers of vascular smooth muscle cells (vSMC) around the aorta. We hypothesize that the process of aortic wall formation is impaired, leading to subsequent rupture and leakage of the blood vessel resulting in death of the embryos. We speculate that misexpression of PDGFRalpha in SMCs causes failure of vSMC recruitment to the aorta.

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Metadaten
Document Type:Article
Language:English
Parent Title (English):Dev Dyn. (Developmental Dynamics)
Volume:238
Issue:5
First Page:1064
Last Page:1072
ISSN:1058-8388
DOI:10.1002/dvdy.21939
Pubmed Id:19334281
Publisher:Wiley
Publication year:2009
Departments, institutes and facilities:Institut für funktionale Gen-Analytik (IfGA)
Dewey Decimal Classification (DDC):600 Technik, Medizin, angewandte Wissenschaften / 610 Medizin und Gesundheit / 610 Medizin und Gesundheit
Entry in this database:2018/07/14

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