Role of Thrombospondin-1 in Mechanotransduction and Development of Thoracic Aortic Aneurysm in Mouse and Humans.

ABSTRACT:

Rationale

Abnormal mechanosensing of smooth muscle cells (SMCs) resulting from the defective elastin-contractile units has been suggested to drive the formation of thoracic aortic aneurysms; however, the precise molecular mechanism has not been elucidated.

Objective

The aim of this study was to identify the crucial mediator(s) involved in abnormal mechanosensing and propagation of biochemical signals during the aneurysm formation and to establish a basis for a novel therapeutic strategy.

Methods and results

We used a mouse model of postnatal ascending aortic aneurysms ( Fbln4^SMKO; termed SMKO [SMC-specific knockout]), in which deletion of Fbln4 (fibulin-4) leads to disruption of the elastin-contractile units caused by a loss of elastic lamina-SMC connection

SUBMITTER: Yamashiro Y 

PROVIDER: S-EPMC6211815 | biostudies-literature | 2018 Aug

REPOSITORIES: biostudies-literature