Current and future treatments of pulmonary arterial hypertension

N Sommer, HA Ghofrani, O Pak… - British journal of …, 2021 - Wiley Online Library
Therapeutic options for pulmonary arterial hypertension (PAH) have increased over the last
decades. The advent of pharmacological therapies targeting the prostacyclin, endothelin …

Matrix remodeling promotes pulmonary hypertension through feedback mechanoactivation of the YAP/TAZ-miR-130/301 circuit

T Bertero, KA Cottrill, YU Lu, CM Haeger… - Cell reports, 2015 - cell.com
Pulmonary hypertension (PH) is a deadly vascular disease with enigmatic molecular origins.
We found that vascular extracellular matrix (ECM) remodeling and stiffening are early and …

Treatment of pulmonary arterial hypertension: recent progress and a look to the future

M Humbert, O Sitbon, C Guignabert… - The Lancet …, 2023 - thelancet.com
Pulmonary arterial hypertension (PAH) is a severe but treatable form of pre-capillary
pulmonary hypertension caused by pulmonary vascular remodelling. As a result of basic …

In pulmonary arterial hypertension, reduced BMPR2 promotes endothelial-to-mesenchymal transition via HMGA1 and its target slug

RK Hopper, JRAJ Moonen, I Diebold, A Cao… - Circulation, 2016 - Am Heart Assoc
Background—We previously reported high-throughput RNA sequencing analyses that
identified heightened expression of the chromatin architectural factor High Mobility Group AT …

Patient-specific iPSC-derived endothelial cells uncover pathways that protect against pulmonary hypertension in BMPR2 mutation carriers

M Gu, NY Shao, S Sa, D Li, V Termglinchan, M Ameen… - Cell stem cell, 2017 - cell.com
In familial pulmonary arterial hypertension (FPAH), the autosomal dominant disease-
causing BMPR2 mutation is only 20% penetrant, suggesting that genetic variation provides …

Immunity and inflammation in pulmonary arterial hypertension: from pathophysiology mechanisms to treatment perspective

R Wang, T Yuan, J Wang, Y Chen, J Zhao, M Li… - Pharmacological …, 2022 - Elsevier
Pulmonary arterial hypertension (PAH) is a severe cardiopulmonary dysfunctional disease,
characterized by progressive vascular remodeling. Inflammation is an increasingly …

Arteriovenous malformations—current understanding of the pathogenesis with implications for treatment

K Schimmel, MK Ali, SY Tan, J Teng, HM Do… - International Journal of …, 2021 - mdpi.com
Arteriovenous malformations are a vascular anomaly typically present at birth, characterized
by an abnormal connection between an artery and a vein (bypassing the capillaries). These …

BMP signaling in vascular biology and dysfunction

AG de Vinuesa, S Abdelilah-Seyfried, P Knaus… - Cytokine & growth factor …, 2016 - Elsevier
The vascular system is critical for developmental growth, tissue homeostasis and repair but
also for tumor development. Bone morphogenetic protein (BMP) signaling has recently …

Caveolar peroxynitrite formation impairs endothelial TRPV4 channels and elevates pulmonary arterial pressure in pulmonary hypertension

Z Daneva, C Marziano, M Ottolini… - Proceedings of the …, 2021 - National Acad Sciences
Recent studies have focused on the contribution of capillary endothelial TRPV4 channels to
pulmonary pathologies, including lung edema and lung injury. However, in pulmonary …

Apelin, Elabela/Toddler, and biased agonists as novel therapeutic agents in the cardiovascular system

P Yang, JJ Maguire, AP Davenport - Trends in pharmacological sciences, 2015 - cell.com
Apelin and its G protein-coupled receptor (GPCR) have emerged as a key signalling
pathway in the cardiovascular system. The peptide is a potent inotropic agent and …