Open AccessModulation of Endothelial Bone Morphogenetic Protein Receptor Type 2 Activity by Vascular Endothelial Growth Factor Receptor 3 in Pulmonary Arterial Hypertension
Author(s) -
Cheol Hwangbo,
HeonWoo Lee,
Hyeseon Kang,
Hyekyung Ju,
David Wiley,
Irinna Papangeli,
Jinah Han,
JunDae Kim,
William P. Dunworth,
Xiaoyue Hu,
Seyoung Lee,
Omar El-Hely,
Avraham Sofer,
Boryeong Pak,
Laura Peterson,
Suzy Comhair,
Eun Mi Hwang,
JaeYong Park,
JeanLéon Thomas,
Victoria L. Bautch,
Serpil C. Erzurum,
Hyung J. Chun,
Suk-Won Jin
Publication year - 2017
Publication title -
circulation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 7.795
H-Index - 607
eISSN - 1524-4539
pISSN - 0009-7322
DOI - 10.1161/circulationaha.116.025390
Subject(s) - bmpr2 , medicine , bone morphogenetic protein , vascular endothelial growth factor b , angiogenesis , microbiology and biotechnology , cancer research , endocrinology , vascular endothelial growth factor a , vascular endothelial growth factor , biology , genetics , gene , vegf receptors
Bone morphogenetic protein (BMP) signaling has multiple roles in the development and function of the blood vessels. In humans, mutations in BMP receptor type 2 (BMPR2), a key component of BMP signaling, have been identified in the majority of patients with familial pulmonary arterial hypertension (PAH). However, only a small subset of individuals with BMPR2 mutation develops PAH, suggesting that additional modifiers of BMPR2 function play an important role in the onset and progression of PAH.
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