المؤلفون: Gittenberger-de Groot, A.C., Hoppenbrouwers, T., Miquerol, L., Kosaka, Y., Poelmann, R.E., Wisse, L.J., Yost, H.J., Jongbloed, M.R.M., Deruiter, M.C., Brunelli, L.

المساهمون: Institut de Biologie du Développement de Marseille (IBDM), Aix Marseille Université (AMU)-Collège de France (CdF (institution))-Centre National de la Recherche Scientifique (CNRS)

المصدر: Developmental Dynamics
Developmental Dynamics, 2016, 245 (11), pp.1107-1123. ⟨10.1002/dvdy.24440.Epub2016Sep18⟩
Developmental Dynamics, Wiley, 2016, 245 (11), pp.1107-1123. ⟨10.1002/dvdy.24440.Epub2016Sep18⟩
Developmental Dynamics, 245(11), 1107-1123

مصطلحات موضوعية: ventricular septal defect, coronary artery hypoplasia, endocardial cushion, cardiovascular system, cardiovascular diseases, heart development, [SDV.BC]Life Sciences [q-bio]/Cellular Biology, myocardial hypoplasia, 14-3-3, mouse, cardiac outflow tract

الوصف: International audience; BACKGROUND:14-3-3ε plays an important role in the maturation of the compact ventricular myocardium by modulating the cardiomyocyte cell cycle via p27kip1 . However, additional cardiac defects are possible given the ubiquitous expression pattern of this protein.RESULTS:Germ line deletion of 14-3-3ε led to malalignment of both the outflow tract (OFT) and atrioventricular (AV) cushions, with resulting tricuspid stenosis and atresia, mitral valve abnormalities, and perimembranous ventricular septal defects (VSDs). We confirmed myocardial non-compaction and detected a spongy septum with muscular VSDs and blebbing of the epicardium. These defects were associated with abnormal patterning of p27kip1 expression in the subendocardial and possibly the epicardial cell populations. In addition to abnormal pharyngeal arch artery patterning, we found deep endocardial recesses and paucity of intramyocardial coronary vasculature as a result of defective coronary plexus remodeling.CONCLUSIONS:The malalignment of both endocardial cushions provides a new explanation for tricuspid and mitral valve defects, while myocardial non-compaction provides the basis for the abnormal coronary vasculature patterning. These abnormalities might arise from p27kip1 dysregulation and a resulting defect in epithelial-to-mesenchymal transformation. These data suggest that 14-3-3ε, in addition to left ventricular non-compaction (LVNC), might be linked to different forms of congenital heart disease (CHD). Developmental Dynamics 245:1107-1123, 2016. © 2016 Wiley Periodicals, Inc.

الوصول الحر: https://explore.openaire.eu/search/publication?articleId=dedup_wf_001::7d4f7b49d182620369a66d381e9e2c05Test
https://hal.science/hal-01444427Test

المؤلفون: Théveniau-Ruissy, M., Pérez-Pomares, J.M., Parisot, P., Baldini, A., Miquerol, L., Kelly, R.G

المساهمون: Institut de Biologie du Développement de Marseille (IBDM), Aix Marseille Université (AMU)-Collège de France (CdF (institution))-Centre National de la Recherche Scientifique (CNRS)

المصدر: ISSN: 1058-8388.

مصطلحات موضوعية: Tbx1, coronary arteries, coronary ostia, heart development, [SDV.BC]Life Sciences [q-bio]/Cellular Biology

الوصف: International audience ; BACKGROUND:Coronary artery (CA) stems connect the ventricular coronary tree with the aorta. Defects in proximal CA patterning are a cause of sudden cardiac death. In mice lacking Tbx1, common arterial trunk is associated with an abnormal trajectory of the proximal left CA. Here we investigate CA stem development in wild-type and Tbx1 null embryos.RESULTS:Genetic lineage tracing reveals that limited outgrowth of aortic endothelium contributes to proximal CA stems. Immunohistochemistry and fluorescent tracer injections identify a periarterial vascular plexus present at the onset of CA stem development. Transplantation experiments in avian embryos indicate that the periarterial plexus originates in mesenchyme distal to the outflow tract. Tbx1 is required for the patterning but not timing of CA stem development and a Tbx1 reporter allele is expressed in myocardium adjacent to the left but not right CA stem. This expression domain is maintained in Sema3c(-/-) hearts with a common arterial trunk and leftward positioned CA. Ectopic myocardial differentiation is observed on the left side of the Tbx1(-/-) common arterial trunk.CONCLUSIONS:A periarterial plexus bridges limited outgrowth of the aortic endothelium with the ventricular plexus during CA stem development. Molecular differences associated with left and right CA stems provide new insights into the etiology of CA patterning defects.

العلاقة: hal-01442525; https://hal.science/hal-01442525Test

الإتاحة: https://doi.org/10.1002/dvdy.24380.Epub2016Jan19Test
https://hal.science/hal-01442525Test