Article
Deficient Circumferential Growth Is the Primary Determinant of Aortic Obstruction Attributable to Partial Elastin Deficiency.
Arteriosclerosis, thrombosis, and vascular biology - 1 May 2017
Jiao Yang, Li Guangxin, Korneva Arina, Caulk Alexander W, Qin Lingfeng, Bersi Matthew R, Li Qingle, Li Wei, Mecham Robert P, Humphrey Jay D, Tellides George
Abstract excerpt
OBJECTIVE: Williams syndrome is characterized by obstructive aortopathy attributable to heterozygous loss of ELN, the gene encoding elastin. Lesions are thought to result primarily from excessive smooth muscle cell (SMC) proliferation and consequent medial expansion, although an initially smaller caliber and increased stiffness of the aorta may contribute to luminal narrowing. The relative contributions of such...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.