Pdgfrα functions in endothelial-derived cells to regulate neural crest cells and the development of the great arteries.
Dis Model Mech
; 10(9): 1101-1108, 2017 09 01.
Article
de En
| MEDLINE
| ID: mdl-28714851
ABSTRACT
Originating as a single vessel emerging from the embryonic heart, the truncus arteriosus must septate and remodel into the aorta and pulmonary artery to support postnatal life. Defective remodeling or septation leads to abnormalities collectively known as conotruncal defects, which are associated with significant mortality and morbidity. Multiple populations of cells must interact to coordinate outflow tract remodeling, and the cardiac neural crest has emerged as particularly important during this process. Abnormalities in the cardiac neural crest have been implicated in the pathogenesis of multiple conotruncal defects, including persistent truncus arteriosus, double outlet right ventricle and tetralogy of Fallot. However, the role of the neural crest in the pathogenesis of another conotruncal abnormality, transposition of the great arteries, is less well understood. In this report, we demonstrate an unexpected role of Pdgfra in endothelial cells and their derivatives during outflow tract development. Loss of Pdgfra in endothelium and endothelial-derived cells results in double outlet right ventricle and transposition of the great arteries. Our data suggest that loss of Pdgfra in endothelial-derived mesenchyme in the outflow tract endocardial cushions leads to a secondary defect in neural crest migration during development.
Mots clés
Texte intégral:
1
Collection:
01-internacional
Base de données:
MEDLINE
Sujet principal:
Artères
/
Récepteur au PDGF alpha
/
Cellules endothéliales
/
Crête neurale
Limites:
Animals
Langue:
En
Journal:
Dis Model Mech
Sujet du journal:
MEDICINA
Année:
2017
Type de document:
Article
Pays d'affiliation:
États-Unis d'Amérique