Journal
AMERICAN JOURNAL OF PATHOLOGY
Volume 180, Issue 3, Pages 1136-1144Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.ajpath.2011.12.006
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Funding
- NIH [HL55374, HL54710, HL089407]
- Swedish Research Council
- LeDucq Foundation
- Swedish Brain Foundation and Hallstens Research Foundation
- Swedish Agency for Innovation Systems
- Swedish Stroke Foundation
- Karolinska Institute
- Ludwig Institute for Cancer Research
- Soderberg Foundation
- Lars Hierta Memorial Foundation
- Ake Wiberg Foundation
- Canadian Institutes of Health Research
- Swedish Cancer Foundation
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Platelet-derived growth factors (PDGFs) and their tyrosine kinase receptors (PDGFRs) are known to play important roles during development of the lungs, central nervous system (CNS), and skeleton and in several diseases. PDGF-C is a ligand for the tyrosine kinase receptor PDGFR alpha. Mutations in the gene encoding PDGF-C have been linked to clefts of the lip and/or palate in humans, and ablation of PDGF-C in 129/Sv background mice results in death during the perinatal period. In this study, we report that ablation of PDGF-C in C57BL/6 mice results in a milder pheno-dymal integrity as well as in normal cerebral vascularization. (Am J Pathol 2012, 180:1136-1144; DOI: 10.1016/j.ajpath.2011.12.006)type than in 129/Sv mice, and we present a phenotypic characterization of PDGF-C deficiency in the adult murine CNS. Multiple congenital defects were observed in the CNS of PDGF-C null C57BL/6 mice, including cerebral vascular abnormalities with abnormal vascular smooth muscle cell coverage. In vivo imaging of mice deficient in PDGF-C also revealed cerebral ventricular abnormalities, such as asymmetry of the lateral ventricles and hypoplasia of the septum, reminiscent of cavum septum pellucidum in humans. We further noted that PDGF-C deficient mice displayed a distorted ependymal lining of the lateral ventricles, and we found evidence of misplaced neurons in the ventricular lining. We conclude that PDGF-C plays a critical role in the development of normal cerebral ventricles and neuroepen-dymal integrity as well as in normal cerebral vascularization. (Am J Pathol 2012, 180:1136-1144; DOI: 10.1016/j.ajpath.2011.12.006)
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