2025
conference-abstract
OpenAlex
Aida Llucià‐Valldeperas, rowan Smal, Jessie van Wezenbeek, Fjodor Bekedam et autres
Background: Pulmonary arterial hypertension (PAH) is a rare disease that originates in the lungs and patients eventually die of right heart failure. A mutation in the Bone Morphogenetic Protein Receptor Type 2 (BMPR2) gene is present in 70% of hereditary PAH patients. …
nl
(code pays fourni par la source)
2025
conference-abstract
OpenAlex
Eszter Tóth, Clarissa Becher, Carolina Janssen Telders, Tobias Neumann et autres
Introduction: Pathogenic BMPR2 variants are a major genetic risk factor for pulmonary arterial hypertension (PAH), though the disease shows incomplete penetrance, suggesting that additional modifiers influence diease onset. While cytokine profiles are altered in PAH, it remains unclear whether unaffected carriers (UCs) …
nl
(code pays fourni par la source)
2025
conference-abstract
OpenAlex
Aida Llucià‐Valldeperas, Jessie van Wezenbeek, Joanne A. Groeneveldt, Rowan Smal et autres
Precapillary pulmonary hypertension (precPH) results in increased right atrial (RA) stretch and pressure. The right atrium is the major source of bone morphogenetic protein 10 (BMP10) in adults. Our aim was to investigate BMP10 expression in the right atrium and peripheral blood …
nl, es
(code pays fourni par la source)
2025
conference-abstract
OpenAlex
Eszter Nóra Tóth, Clarissa Becher, Tobias Neumann, Jessie van Wezenbeek et autres
Introduction: Pathogenic variants in the BMPR2 gene are a major genetic risk factor for hereditary pulmonary arterial hypertension (PAH). PAH exhibits incomplete penetrance, suggesting other modifying factors may influence disease development. Previous studies have shown that inflammatory cytokines such as IL-10, CXCL-10, …
nl
(code pays fourni par la source)
2025
conference-abstract
OpenAlex
Jessie van Wezenbeek, Aida Llucià‐Valldeperas, Anton Vonk Noordegraaf, Harm Jan Bogaard et autres
Introduction: Pulmonary arterial hypertension (PAH) is characterized by right ventricular (RV) overload. N-terminal Brain Natriuretic Peptides (NT-proBNP) and Atrial Natriuretic Peptides (ANP) are secreted from cardiomyocytes upon stretch. Aim: To investigate the association between ANP and NTproBNP and pressure overload in blood, …
nl
(code pays fourni par la source)
Accès ouvert
2025
article
OpenAlex
Aida Llucià‐Valldeperas, Jessie van Wezenbeek, Joanne A. Groeneveldt, Rowan Smal et autres
AIMS: Pre-capillary pulmonary hypertension (precPH) results in increased right atrial (RA) stretch and pressure. The right atrium is the major source of bone morphogenetic protein 10 (BMP10) in adults, primarily produced by RA cardiomyocytes. The aim of this study was to investigate …
us, nl, es
(code pays fourni par la source)
Accès ouvert
2025
article
OpenAlex
Nadia Bernardi, Beau Neep, Silvano Garibaldi, Eleonora Bianconi et autres
Long non-coding RNA (lncRNA) may be involved in dysfunction of pulmonary artery endothelial cells (PAEC) and, thus, in pulmonary arterial hypertension (PAH) pathobiology. We screened the RNA expression profile of commercial human PAEC (hPAEC) exposed to increased hydrostatic pressure, and found that …
it, nl
(code pays fourni par la source)
Accès ouvert
2025
preprint
OpenAlex
Marius Wits, Nerea Gomez-Suarez, Nicole Farfán, Fjodor Bekedam et autres
Abstract Background Fibrodysplasia ossificans progressiva (FOP) is caused by an activating mutation (p.R206H) in the type I BMP receptor ALK2, leading to heterotopic ossification (HO) in soft connective tissues. While aberrant Activin A-induced SMAD signaling is central in FOP pathogenesis, global signaling …
nl, es
(code pays fourni par la source)
2024
conference-abstract
OpenAlex
Aida Llucià‐Valldeperas, Jessie van Wezenbeek, Rowan Smal, Fjodor Bekedam et autres
Introduction: A mutation in the Bone Morphogenetic Protein Receptor Type 2 ( BMPR2 ) gene is in 70% of hereditary pulmonary arterial hypertension (hPAH) patients the causative mutation of the disease. Previous work demonstrated that right ventricular function is more impaired in …
nl
(code pays fourni par la source)
2024
conference-abstract
OpenAlex
Aida Llucià‐Valldeperas, Jessie van Wezenbeek, Joanne A. Groeneveldt, Gonzalo Sánchez‐Duffhues et autres
Introduction: Precapillary pulmonary hypertension (precPH) leads to increased right atrial (RA) stretch and pressure. The right atrium is the major source of bone morphogenetic protein 10 (BMP10) in adults. Aim: This study aims to investigate BMP10 in relation to RA dilatation and …
nl
(code pays fourni par la source)
Accès ouvert
2024
article
OpenAlex
Stine Andersen, Julie Birkmose Axelsen, Anders Hammer Nielsen-Kudsk, Janne Schwab et autres
Abstract Activation of the sympathetic nervous system is observed in pulmonary arterial hypertension patients. This study investigates whether inhibiting the conversion of dopamine into noradrenaline by dopamine β‐hydroxylase (DβH) inhibition with BIA 21‐5337 improved right ventricular (RV) function or remodeling in pressure …
dk, pt
(code pays fourni par la source)
Accès ouvert
2024
article
OpenAlex
Julie Birkmose Axelsen, Stine Andersen, Steffen Ringgaard, Rowan Smal et autres
Different rat strains are used in various animal models of pulmonary hypertension and right ventricular (RV) failure. No systematic assessment has been made to test differences in RV response to pressure overload between rat strains. We compared RV adaptation to pulmonary trunk …
dk, nl
(code pays fourni par la source)