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Platelet-derived growth factor receptor alpha subunit deleted Patch mouse exhibits severe cardiovascular
G C Schatteman1, S T Motley, E L Effmann
1Department of Pathology, University of Washington, Seattle 98195, USA.
Teratology
|June 1, 1995
Summary
Deletion of the platelet-derived growth factor receptor alpha subunit (alpha PDGFR) gene in mice causes severe cardiovascular defects. This highlights alpha PDGFR
Area of Science:
- Developmental Biology
- Cardiovascular Science
- Genetics
Background:
- Platelet-derived growth factor receptor alpha subunit (alpha PDGFR) plays a role in mammalian development.
- Patch (Ph) mice, lacking functional alpha PDGFR, exhibit developmental abnormalities.
Purpose of the Study:
- To investigate the specific role of alpha PDGFR in murine cardiovascular development.
- To correlate alpha PDGFR expression patterns with observed cardiovascular defects in Ph/Ph embryos.
Main Methods:
- In situ hybridization to track alpha PDGFR mRNA expression in embryonic cardiovascular tissues.
- Comparative analysis of cardiovascular morphology in wild-type and Ph/Ph mutant embryos.
Main Results:
- Ph/Ph embryos display severe dysmorphia in both mesodermal and neural crest-derived cardiovascular components.
- Defects are most pronounced in structures with high and sustained alpha PDGFR expression.
- Vessels show reduced smooth muscle cells and fragility; heart exhibits thin myocardium, malformed valves, and septal defects.
Conclusions:
- Alpha PDGFR is essential for normal cardiovascular development, impacting both mesodermal and neural crest contributions.
- The observed cardiovascular defects suggest a functional ablation of cranial neural crest cells in Ph/Ph mutants.
- These findings underscore the critical requirement of alpha PDGFR for vascular and cardiac structure integrity.