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 …

Cellular and molecular basis of pulmonary arterial hypertension

NW Morrell, S Adnot, SL Archer, J Dupuis… - Journal of the American …, 2009 - jacc.org
Pulmonary arterial hypertension (PAH) is caused by functional and structural changes in the
pulmonary vasculature, leading to increased pulmonary vascular resistance. The process of …

A novel channelopathy in pulmonary arterial hypertension

L Ma, D Roman-Campos, ED Austin… - … England Journal of …, 2013 - Mass Medical Soc
Background Pulmonary arterial hypertension is a devastating disease with high mortality.
Familial cases of pulmonary arterial hypertension are usually characterized by autosomal …

Basic science of pulmonary arterial hypertension for clinicians: new concepts and experimental therapies

SL Archer, EK Weir, MR Wilkins - Circulation, 2010 - Am Heart Assoc
Pulmonary arterial hypertension (PAH) is a syndrome in which pulmonary arterial
obstruction increases pulmonary vascular resistance, which leads to right ventricular (RV) …

Pulmonary arterial hypertension: a current perspective on established and emerging molecular genetic defects

RD Machado, L Southgate, CA Eichstaedt… - Human …, 2015 - Wiley Online Library
Pulmonary arterial hypertension (PAH) is an often fatal disorder resulting from several
causes including heterogeneous genetic defects. While mutations in the bone …

Genetics and genomics of pulmonary arterial hypertension

RD Machado, O Eickelberg, CG Elliott… - Journal of the American …, 2009 - jacc.org
Pulmonary arterial hypertension (PAH) is a rare disorder that may be hereditable (HPAH),
idiopathic (IPAH), or associated with either drug-toxin exposures or other medical …

Pathophysiology and pathogenic mechanisms of pulmonary hypertension: role of membrane receptors, ion channels, and Ca2+ signaling

A Balistrieri, A Makino, JXJ Yuan - Physiological reviews, 2023 - journals.physiology.org
The pulmonary circulation is a low-resistance, low-pressure, and high-compliance system
that allows the lungs to receive the entire cardiac output. Pulmonary arterial pressure is a …

Pathogenic mechanisms of pulmonary arterial hypertension

SY Chan, J Loscalzo - Journal of molecular and cellular cardiology, 2008 - Elsevier
Pulmonary arterial hypertension (PAH) is a complex disease that causes significant
morbidity and mortality and is clinically characterized by an increase in pulmonary vascular …

Mitochondria in vascular health and disease

P Dromparis, ED Michelakis - Annual review of physiology, 2013 - annualreviews.org
The eukaryote's mitochondrial network is perhaps the cell's most sophisticated and dynamic
responsive sensing system. Integrating metabolic, oxygen, or danger signals with inputs …

The genetics of pulmonary arterial hypertension

ED Austin, JE Loyd - Circulation research, 2014 - Am Heart Assoc
Pulmonary arterial hypertension (PAH) is a progressive and fatal disease for which there is
an ever-expanding body of genetic and related pathophysiological information on disease …