Primary pulmonary hypertension is associated with reduced pulmonary vascular expression of type II bone morphogenetic protein receptor

C Atkinson, S Stewart, PD Upton, R Machado… - Circulation, 2002 - Am Heart Assoc
Background— Mutations in the type II receptor for bone morphogenetic protein (BMPR-II), a
receptor member of the transforming growth factor-β (TGF-β) superfamily, underlie many …

Altered Growth Responses of Pulmonary Artery Smooth Muscle Cells From Patients With Primary Pulmonary Hypertension to Transforming Growth Factor-β1 and …

NW Morrell, X Yang, PD Upton, KB Jourdan… - Circulation, 2001 - Am Heart Assoc
Background Mutations in the type II receptor for bone morphogenetic protein (BMPR-II), a
receptor member of the transforming growth factor-β (TGF-β) superfamily, underlie many cases …

Selective enhancement of endothelial BMPR-II with BMP9 reverses pulmonary arterial hypertension

…, SD Moore, KM Drake, MA Aldred, PB Yu, PD Upton… - Nature medicine, 2015 - nature.com
Genetic evidence implicates the loss of bone morphogenetic protein type II receptor (BMPR-II)
signaling in the endothelium as an initiating factor in pulmonary arterial hypertension (PAH…

ETA and ETB Receptors Modulate the Proliferation of Human Pulmonary Artery Smooth Muscle Cells

N Davie, SJ Haleen, PD Upton, JM Polak… - American journal of …, 2002 - atsjournals.org
We determined the distribution of ET A and ET B receptors in pulmonary arteries from
pulmonary hypertensive patients and control subjects, using in vitro autoradiography, and …

Dysfunctional Smad signaling contributes to abnormal smooth muscle cell proliferation in familial pulmonary arterial hypertension

…, M Southwood, N Rudarakanchana, PD Upton… - Circulation …, 2005 - Am Heart Assoc
Mutations in the bone morphogenetic protein type II receptor gene (BMPR2) are the major
genetic cause of familial pulmonary arterial hypertension (FPAH). Although smooth muscle …

[HTML][HTML] Identification of rare sequence variation underlying heritable pulmonary arterial hypertension

…, N Soranzo, A Lawrie, PD Upton… - 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 …

[PDF][PDF] BMP9 mutations cause a vascular-anomaly syndrome with phenotypic overlap with hereditary hemorrhagic telangiectasia

…, J McDonald, B O'Fallon, PD Upton… - The American Journal of …, 2013 - cell.com
Hereditary hemorrhagic telangiectasia (HHT), the most common inherited vascular disorder,
is caused by mutations in genes involved in the transforming growth factor beta (TGF-β) …

A role for miR-145 in pulmonary arterial hypertension: evidence from mouse models and patient samples

…, KM Mair, JD McClure, M Southwood, P Upton… - Circulation …, 2012 - Am Heart Assoc
Rationale: Despite improved understanding of the underlying genetics, pulmonary arterial
hypertension (PAH) remains a severe disease. Extensive remodeling of small pulmonary …

Altered bone morphogenetic protein and transforming growth factor-β signaling in rat models of pulmonary hypertension: potential for activin receptor-like kinase-5 …

L Long, A Crosby, X Yang, M Southwood, PD Upton… - Circulation, 2009 - Am Heart Assoc
Background— Recent genetic studies have highlighted the role of the bone morphogenetic
protein (BMP)/transforming growth factor (TGF)-β signaling pathways in the pathogenesis of …

Circulating adrenomedullin does not regulate systemic blood pressure but increases plasma prolactin after intravenous infusion in humans: a pharmacokinetic study

K Meeran, D O'Shea, PD Upton… - The Journal of …, 1997 - academic.oup.com
Adrenomedullin has been proposed to be a circulating hormone regulating systemic and
pulmonary blood pressure. A potential therapeutic role in the management of pulmonary …