Pulmonary arterial hypertension: pathogenesis and clinical management

T Thenappan, ML Ormiston, JJ Ryan, SL Archer - Bmj, 2018 - bmj.com
Pulmonary hypertension is defined as a resting mean pulmonary artery pressure of 25 mm
Hg or above. This review deals with pulmonary arterial hypertension (PAH), a type of …

Signaling receptors for TGF-β family members

CH Heldin, A Moustakas - Cold Spring Harbor …, 2016 - cshperspectives.cshlp.org
Transforming growth factor β (TGF-β) family members signal via heterotetrameric complexes
of type I and type II dual specificity kinase receptors. The activation and stability of the …

Epidemiology and treatment of pulmonary arterial hypertension

EMT Lau, E Giannoulatou, DS Celermajer… - Nature Reviews …, 2017 - nature.com
In the past 2 decades, major changes have occurred in the epidemiological and treatment
landscape of pulmonary arterial hypertension (PAH). Previously regarded as a disease of …

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 …

ACCF/AHA 2009 expert consensus document on pulmonary hypertension: a report of the American College of Cardiology Foundation task force on expert consensus …

Writing Committee Members, VV McLaughlin… - Circulation, 2009 - Am Heart Assoc
ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension | Circulation Science
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EIF2AK4 mutations cause pulmonary veno-occlusive disease, a recessive form of pulmonary hypertension

M Eyries, D Montani, B Girerd, C Perret, A Leroy… - Nature …, 2014 - nature.com
Pulmonary veno-occlusive disease (PVOD) is a rare and devastating cause of pulmonary
hypertension that is characterized histologically by widespread fibrous intimal proliferation of …

[HTML][HTML] Preparing for the first breath: genetic and cellular mechanisms in lung development

EE Morrisey, BLM Hogan - Developmental cell, 2010 - cell.com
The mammalian respiratory system—the trachea and the lungs—arises from the anterior
foregut through a sequence of morphogenetic events involving reciprocal endodermal …

Cellular and molecular pathobiology of pulmonary arterial hypertension

M Humbert, NW Morrell, SL Archer… - Journal of the American …, 2004 - jacc.org
Pulmonary arterial hypertension (PAH) has a multifactorial pathobiology. Vasoconstriction,
remodeling of the pulmonary vessel wall, and thrombosis contribute to increased pulmonary …

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 …

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 …