Codependence of Bone Morphogenetic Protein Receptor 2 and Transforming Growth Factor-β in Elastic Fiber Assembly and Its Perturbation in Pulmonary Arterial Hypertension | Zendy

Nancy F. Tojais | Zendy; Aiqin Cao | Zendy; YingJu Lai | Zendy; Lingli Wang | Zendy; Pin-I Chen | Zendy; Miguel A. Alejandre Alcázar | Zendy; Vinicio A. de Jesús Pérez | Zendy; Rachel K. Hopper | Zendy; Christopher J. Rhodes | Zendy; Matthew A. Bill | Zendy; Lynn Y. Sakai | Zendy; Marlene Rabinovitch | Zendy

Open Access

Codependence of Bone Morphogenetic Protein Receptor 2 and Transforming Growth Factor-β in Elastic Fiber Assembly and Its Perturbation in Pulmonary Arterial Hypertension

Author(s) -

Nancy F. Tojais,

Aiqin Cao,

YingJu Lai,

Lingli Wang,

Pin-I Chen,

Miguel A. Alejandre Alcázar,

Vinicio A. de Jesús Pérez,

Rachel K. Hopper,

Christopher J. Rhodes,

Matthew A. Bill,

Lynn Y. Sakai,

Marlene Rabinovitch

Publication year - 2017

Publication title -

arteriosclerosis thrombosis and vascular biology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 3.007

H-Index - 270

eISSN - 1524-4636

pISSN - 1079-5642

DOI - 10.1161/atvbaha.117.309696

Subject(s) - elastin , bmpr2 , tropoelastin , fibrillin , elastic fiber , fibroblast , bone morphogenetic protein , transforming growth factor , chemistry , medicine , receptor , endocrinology , microbiology and biotechnology , pathology , extracellular matrix , biology , biochemistry , in vitro , gene

We determined in patients with pulmonary arterial (PA) hypertension (PAH) whether in addition to increased production of elastase by PA smooth muscle cells previously reported, PA elastic fibers are susceptible to degradation because of their abnormal assembly.

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