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Altered expression of dystrophin within the thoracic aorta in coarctation

Published online by Cambridge University Press:  14 April 2005

Matteo Vatta ,
Anthony C. Chang  and
Colin J. McMahon
Matteo Vatta
Affiliation:
Department of Pediatrics (Cardiology), Baylor College of Medicine, and Texas Children's Hospital, Houston, Texas, United States of America Department of Reproductive and Developmental Sciences, University of Trieste, Trieste, Italy
Anthony C. Chang
Affiliation:
Department of Pediatrics (Cardiology), Baylor College of Medicine, and Texas Children's Hospital, Houston, Texas, United States of America
Colin J. McMahon
Affiliation:
Department of Pediatrics (Cardiology), Baylor College of Medicine, and Texas Children's Hospital, Houston, Texas, United States of America
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Abstract

Although persisting endothelial dysfunction has been established in the vasculature of patients following surgical repair of coarctation, it is unknown whether there are alterations in the cytoskeleton of the aorta in such patients. We compared staining of N-terminus dystrophin in the smooth muscle of the aortic wall of a patient with coarctation to that in a patient without coarctation, the latter undergoing surgical treatment of a double aortic arch. There was a marked difference in the pattern of expression of dystrophin between the two, with the coarcted specimen demonstrating marked fragmentation but normal intensity of staining. As far as we are aware, ours is the first report to demonstrate the presence of dystrophin in the smooth muscle of the aorta. Alterations in the cytoskeletal structure may account for underlying aberrations in endothelial function in such patients, and is a topic that warrants further investigation.

Keywords

Aortacytoskeletonendothelial function
Type
Brief Report
Information
Cardiology in the Young , Volume 15 , Issue 1 , January 2005 , pp. 73 - 74
Copyright
2005 Cambridge University Press

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References

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