Endothelial cells in the pathogenesis of pulmonary arterial hypertension

CE Evans, ND Cober, Z Dai… - European Respiratory …, 2021 - Eur Respiratory Soc
Pulmonary arterial hypertension (PAH) is a devastating disease that involves pulmonary
vasoconstriction, small vessel obliteration, large vessel thickening and obstruction, and …

Inflammation and immunity in the pathogenesis of pulmonary arterial hypertension

M Rabinovitch, C Guignabert, M Humbert… - Circulation …, 2014 - Am Heart Assoc
This review summarizes an expanding body of knowledge indicating that failure to resolve
inflammation and altered immune processes underlie the development of pulmonary arterial …

TNFα drives pulmonary arterial hypertension by suppressing the BMP type-II receptor and altering NOTCH signalling

LA Hurst, BJ Dunmore, L Long, A Crosby… - Nature …, 2017 - nature.com
Heterozygous germ-line mutations in the bone morphogenetic protein type-II receptor
(BMPR-II) gene underlie heritable pulmonary arterial hypertension (HPAH). Although …

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 …

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 …

Ectopic upregulation of membrane-bound IL6R drives vascular remodeling in pulmonary arterial hypertension

Y Tamura, C Phan, L Tu, M Le Hiress… - The Journal of …, 2018 - Am Soc Clin Investig
Pulmonary arterial hypertension (PAH) is characterized by a progressive accumulation of
pulmonary artery smooth muscle cells (PA-SMCs) in pulmonary arterioles leading to the …

The cancer theory of pulmonary arterial hypertension

O Boucherat, G Vitry, I Trinh, R Paulin… - Pulmonary …, 2017 - journals.sagepub.com
Pulmonary arterial hypertension (PAH) remains a mysterious killer that, like cancer, is
characterized by tremendous complexity. PAH development occurs under sustained and …

BMPR2 preserves mitochondrial function and DNA during reoxygenation to promote endothelial cell survival and reverse pulmonary hypertension

I Diebold, JK Hennigs, K Miyagawa, CG Li, NP Nickel… - Cell metabolism, 2015 - cell.com
Mitochondrial dysfunction, inflammation, and mutant bone morphogenetic protein receptor 2
(BMPR2) are associated with pulmonary arterial hypertension (PAH), an incurable disease …

Role of inflammatory cell subtypes in heart failure

D Strassheim, EC Dempsey… - Journal of …, 2019 - Wiley Online Library
Inflammation is a well‐known feature of heart failure. Studies have shown that while some
inflammation is required for repair during injury and is protective, prolonged inflammation …

Chronic inflammation within the vascular wall in pulmonary arterial hypertension: more than a spectator

A Huertas, L Tu, M Humbert… - Cardiovascular …, 2020 - academic.oup.com
This review seeks to provide an update of preclinical findings and available clinical data on
the chronic persistent inflammation and its direct role on the pulmonary arterial hypertension …