Absence of TGFβ signaling in embryonic vascular smooth muscle leads to reduced lysyl oxidase expression,impaired elastogenesis,and aneurysm |
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Authors: | Bibha Choudhary Jingjing Zhou Peng Li Simmy Thomas Vesa Kaartinen Henry M Sucov |
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Institution: | 1. Institute for Genetic Medicine, University of Southern California Keck School of Medicine, Los Angeles, California;2. Developmental Biology Program, Department of Pathology, The Saban Research Institute of Childrens Hospital Los Angeles, Los Angeles, California |
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Abstract: | To address the requirement for TGFβ signaling in the formation and maintenance of the vascular matrix, we employed lineage‐specific mutation of the type II TGFβ receptor gene (Tgfbr2) in vascular smooth muscle precursors in mice. In both neural crest‐ and mesoderm‐derived smooth muscle, absence of TGFβ receptor function resulted in a poorly organized vascular elastic matrix in late‐stage embryos which was prone to dilation and aneurysm. This defect represents a failure to initiate formation of the elastic matrix, rather than a failure to maintain a preexisting matrix. In mutant tissue, lysyl oxidase expression was substantially reduced, which may contribute to the observed pathology. genesis 47:115–121, 2009. © 2009 Wiley‐Liss, Inc. |
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Keywords: | TGFβ elastogenesis aneurysm lysyl oxidase fibrillin Marfan syndrome Loeys‐Dietz syndrome mouse mutant |
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