Sox17 is required for normal pulmonary vascular morphogenesis. Dev Biol 2014 Mar 01;387(1):109-20
Date
01/15/2014Pubmed ID
24418654Pubmed Central ID
PMC4422074DOI
10.1016/j.ydbio.2013.11.018Scopus ID
2-s2.0-84893783089 (requires institutional sign-in at Scopus site) 51 CitationsAbstract
The SRY-box containing transcription factor Sox17 is required for endoderm formation and vascular morphogenesis during embryonic development. In the lung, Sox17 is expressed in mesenchymal progenitors of the embryonic pulmonary vasculature and is restricted to vascular endothelial cells in the mature lung. Conditional deletion of Sox17 in splanchnic mesenchyme-derivatives using Dermo1-Cre resulted in substantial loss of Sox17 from developing pulmonary vascular endothelial cells and caused pulmonary vascular abnormalities before birth, including pulmonary vein varices, enlarged arteries, and decreased perfusion of the microvasculature. While survival of Dermo1-Cre;Sox17Δ/Δ mice (herein termed Sox17Δ/Δ) was unaffected at E18.5, most Sox17Δ/Δ mice died by 3 weeks of age. After birth, the density of the pulmonary microvasculature was decreased in association with alveolar simplification, biventricular cardiac hypertrophy, and valvular regurgitation. The severity of the postnatal cardiac phenotype was correlated with the severity of pulmonary vasculature abnormalities. Sox17 is required for normal formation of the pulmonary vasculature and postnatal cardiovascular homeostasis.
Author List
Lange AW, Haitchi HM, LeCras TD, Sridharan A, Xu Y, Wert SE, James J, Udell N, Thurner PJ, Whitsett JAMESH terms used to index this publication - Major topics in bold
AnimalsArteries
Cell Differentiation
Endothelial Cells
Gene Deletion
HMGB Proteins
Lung
Mesoderm
Mice
Mice, Inbred C57BL
Mice, Knockout
Pulmonary Veins
Repressor Proteins
SOXF Transcription Factors
Twist-Related Protein 1