GSE135312 RNA-seq analysis of CRISPR/Cas9 generated human BMPR2 deficient endothelial cell lines harboring mutations characteristic for hereditary pulmonary hypertension (HPAH)

Contributors : Christian Hiepen ; Jerome Jatzlau ; Petra KnausSeries Type : Expression profiling by high throughput sequencingOrganism : Homo sapiensBalanced TGF β/BMP-signalling is essential for tissue formation and homeostasis. While gain in TGFβ signaling is often found in diseases, the underlying cellular mechanisms remain poorly defined. Here we show, that the receptor BMPR2 serves as a central gatekeeper of this balance, highlighted by its deregulati on in diseases such as pulmonary arterial hypertension (PAH). We show that BMPR2-deficient cells express genes indicative for altered biophysical properties including up-regulation of ECM proteins and integrins.
Source: GEO: Gene Expression Omnibus - Category: Genetics & Stem Cells Tags: Expression profiling by high throughput sequencing Homo sapiens Source Type: research

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AbstractPulmonary arterial hypertension is a rare disease, with drug-induced causes even more uncommon, accounting for only 10% of cases in large registry series. Predisposing factors for drug-induced PAH have not been completely defined. This review summarizes drugs with definite, possible, or likely association to pulmonary hypertension and possible mechanisms involved in the occurrence of pulmonary hypertension. Controversies on mechanisms and on their role in pathophysiology were also shown. The possible synergism between drug abuse and HIV was discussed and the possible interactions of antiretroviral therapy in HIV su...
Source: Cardiovascular Drugs and Therapy - Category: Cardiology Source Type: research
Between 16 and 51% of chronic thromboembolic pulmonary hypertension (CTEPH) patients will have residual pulmonary hypertension (PH) after pulmonary endarterectomy (PEA). Whether residual PH is related to remaining (sub-)segmental macrovascular lesions or to microvascular disease is unknown. New imaging techniques can provide detailed information about (sub-)segmental pulmonary arteries and parenchymal perfusion. The aim of this study was to describe the prevalence after PEA of remaining (sub-)segmental vascular lesions on ECG-gated CT pulmonary angiography (CTPA) and parenchymal hypoperfusion on MR and to relate these imag...
Source: The Journal of Heart and Lung Transplantation - Category: Transplant Surgery Authors: Tags: Original Clinical Science Source Type: research
Congenital diaphragmatic hernia (CDH) is a complex disease that causes severe hypoxemic respiratory failure and significant morbidity and mortality.1 In the most severe cases, CDH is characterized by lung hypoplasia, severe pulmonary hypertension (PH), and cardiac dysfunction.2 Despite many advances in therapeutic options for CDH, the related PH and poor cardiac function often remain refractory to medical interventions, such as inhaled nitric oxide (iNO) therapy, and persist as the most common indications for extracorporeal membrane oxygenation therapy (ECMO) in the newborn period.
Source: The Journal of Pediatrics - Category: Pediatrics Authors: Tags: Editorials Source Type: research
Conclusions Patients with precapillary PH related to SCD have a poor prognosis. Thrombotic lesions appear as a major component of PH related to SCD, more frequently in SC patients.
Source: European Respiratory Journal - Category: Respiratory Medicine Authors: Tags: Pulmonary vascular disease Original Articles: Pulmonary vascular disease Source Type: research
Abstract Pulmonary endarterectomy is a curative procedure for chronic thromboembolic Pulmonary Hypertension. As usual, cardiopulmonary bypass circuit is required. However, there are several complications attributed to extracorporeal circulation. Hemodilution, systemic inflammatory response syndrome and leukocyte sequestration are circulation related complications. The severe forms include Acute Respiratory Distress Syndrome, Acute Lung Injury, myocardial stunning, dysfunction of the right ventricle, coagulopathy, postoperative stroke or renal dysfunction. In this case report, we aimed to give information about perioperativ...
Source: Revista Brasileira de Cirurgia Cardiovascular - Category: Cardiovascular & Thoracic Surgery Source Type: research
Abstract Pulmonary endarterectomy is a curative procedure for chronic thromboembolic Pulmonary Hypertension. As usual, cardiopulmonary bypass circuit is required. However, there are several complications attributed to extracorporeal circulation. Hemodilution, systemic inflammatory response syndrome and leukocyte sequestration are circulation related complications. The severe forms include Acute Respiratory Distress Syndrome, Acute Lung Injury, myocardial stunning, dysfunction of the right ventricle, coagulopathy, postoperative stroke or renal dysfunction. In this case report, we aimed to give information about perioperativ...
Source: Revista Brasileira de Cirurgia Cardiovascular - Category: Cardiovascular & Thoracic Surgery Source Type: research
Abstract Pulmonary endarterectomy is a curative procedure for chronic thromboembolic Pulmonary Hypertension. As usual, cardiopulmonary bypass circuit is required. However, there are several complications attributed to extracorporeal circulation. Hemodilution, systemic inflammatory response syndrome and leukocyte sequestration are circulation related complications. The severe forms include Acute Respiratory Distress Syndrome, Acute Lung Injury, myocardial stunning, dysfunction of the right ventricle, coagulopathy, postoperative stroke or renal dysfunction. In this case report, we aimed to give information about perioperativ...
Source: Revista Brasileira de Cirurgia Cardiovascular - Category: Cardiovascular & Thoracic Surgery Source Type: research
Persistent pulmonary hypertension of the newborn (PPHN) can complicate the clinical course of neonates born term and preterm with acute hypoxemic respiratory failure (HRF) and contributes to high morbidity and mortality.1,2 In a study of infants born at term and near-term, PPHN occurred in approximately 2 per 1000 live births.3 In contrast, the prevalence of PPHN among 12  954 infants born extremely preterm from a Japanese cohort was reported at 8.1%, with the trend increasing over the past decade due to increased survival of infants with extremely low birth weight and the growing awareness of PPHN in infants born preterm.
Source: The Journal of Pediatrics - Category: Pediatrics Authors: Tags: Workshop/Symposium Summary Source Type: research
ConclusionThe results of the this study confirm the important role of MDCTA in the evaluation of vascular, cardiac and parenchymal findings in the patients with CTEPH and identifying patients that would most benefit from surgical treatment by visualization of the segmental and subsegmental branches of the pulmonary arteries.
Source: The Clinical Respiratory Journal - Category: Respiratory Medicine Authors: Tags: ORIGINAL ARTICLE Source Type: research
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Source: Journal of Maternal-Fetal and Neonatal Medicine - Category: Perinatology & Neonatology Authors: Source Type: research
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