Molecular genetic framework underlying pulmonary arterial hypertension

L Southgate, RD Machado, S Gräf… - Nature Reviews …, 2020 - nature.com
Pulmonary arterial hypertension (PAH) is a rare, progressive disorder typified by occlusion
of the pulmonary arterioles owing to endothelial dysfunction and uncontrolled proliferation of …

Bone morphogenetic protein receptor signal transduction in human disease

MC Gomez‐Puerto, PV Iyengar… - The journal of …, 2019 - Wiley Online Library
Bone morphogenetic proteins (BMPs) are secreted cytokines that were initially discovered
on the basis of their ability to induce bone. Several decades of research have now …

BMPR2 mutations and survival in pulmonary arterial hypertension: an individual participant data meta-analysis

JDW Evans, B Girerd, D Montani, XJ Wang… - The lancet Respiratory …, 2016 - thelancet.com
Background Mutations in the gene encoding the bone morphogenetic protein receptor type II
(BMPR2) are the commonest genetic cause of pulmonary arterial hypertension (PAH) …

Identification of rare sequence variation underlying heritable pulmonary arterial hypertension

S Gräf, M Haimel, M Bleda, C Hadinnapola… - Nature …, 2018 - nature.com
Pulmonary arterial hypertension (PAH) is a rare disorder with a poor prognosis. Deleterious
variation within components of the transforming growth factor-β pathway, particularly the …

Sotatercept analog suppresses inflammation to reverse experimental pulmonary arterial hypertension

SR Joshi, J Liu, T Bloom, E Karaca Atabay, TH Kuo… - Scientific reports, 2022 - nature.com
Sotatercept is an activin receptor type IIA-Fc (ActRIIA-Fc) fusion protein that improves
cardiopulmonary function in patients with pulmonary arterial hypertension (PAH) by …

Targeting vascular remodeling to treat pulmonary arterial hypertension

AAR Thompson, A Lawrie - Trends in molecular medicine, 2017 - cell.com
Pulmonary arterial hypertension (PAH) describes a group of conditions with a common
hemodynamic phenotype of increased pulmonary artery pressure, driven by progressive …

The role of bone morphogenetic protein signaling in vascular calcification

P Yang, L Troncone, ZM Augur, SSJ Kim, ME McNeil… - Bone, 2020 - Elsevier
Vascular calcification is associated with atherosclerosis, chronic kidney disease, and
diabetes, and results from processes resembling endochondral or intramembranous …

ERK/Drp1‐dependent mitochondrial fission contributes to HMGB1‐induced autophagy in pulmonary arterial hypertension

W Feng, J Wang, X Yan, Q Zhang, L Chai… - Cell …, 2021 - Wiley Online Library
Objectives High‐mobility group box‐1 (HMGB1) and aberrant mitochondrial fission
mediated by excessive activation of GTPase dynamin‐related protein 1 (Drp1) have been …

[HTML][HTML] Bone morphogenetic protein receptors: Structure, function and targeting by selective small molecule kinase inhibitors

G Sanchez-Duffhues, E Williams, MJ Goumans… - Bone, 2020 - Elsevier
Bone morphogenetic proteins (BMPs) are secreted cytokines that control the fate and
function of many different cell types. They exert their cellular responses via heteromeric …

Bone morphogenetic proteins in vascular homeostasis and disease

MJ Goumans, A Zwijsen… - Cold Spring Harbor …, 2018 - cshperspectives.cshlp.org
It is well established that control of vascular morphogenesis and homeostasis is regulated
by vascular endothelial growth factor (VEGF), fibroblast growth factor (FGF), Delta-like 4 …