Idiopathic pulmonary arterial hypertension
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
We have previously demonstrated that low-dose lipopolysaccharide (LPS) is a potent stimulus for the development of PAH in the context of a genetic PAH mouse model of BMPR2 dysfunction.
|
30160594 |
2020 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Pulmonary arterial hypertension is related to mutations in the bone morphogenetic protein receptor type 2, pulmonary vascular dysfunction and is increasingly recognized as a systemic disease.
|
30632900 |
2020 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
Biomarker
|
disease |
BEFREE |
Pulmonary arterial hypertension (PAH) is characterized by pulmonary arterial endothelial cell (PAEC) dysfunction and apoptosis, pulmonary arterial smooth muscle cell (PASMC) proliferation, inflammation, vasoconstriction, and metabolic disturbances that include disrupted bone morphogenetic protein receptor (BMPR2)-peroxisome proliferator-activated receptor gamma (PPARγ) axis and DNA damage.
|
31815758 |
2020 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Documentation of the RNF213 p.Arg4810Lys variant, as well as already known pathogenic genes, such as BMPR2, can provide clinically relevant information for therapeutic strategies, leading to a personalized approach for the treatment of PAH.
|
31542298 |
2020 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
Biomarker
|
disease |
BEFREE |
Since the identification of BMPR2, which encodes a receptor in the transforming growth factor-β superfamily, the development of high-throughput sequencing approaches to identify novel causal genes has substantially advanced our understanding of the molecular genetics of PAH.
|
31406341 |
2020 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
Biomarker
|
disease |
BEFREE |
Altogether, this study sheds light on the basic mechanisms of BMPR2 degradation and highlights a crucial role for autophagy in PAH.© 2019 The Authors.
|
31257577 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
BMPR2 and FHIT expression were reduced in patients with PAH.
|
30107138 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Even in the absence of BMPR2 mutations, increased transforming growth factor (TGF)β receptor signalling and decreased BMPRII signalling have been shown to contribute to PAH pathogenesis.
|
30529762 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
Biomarker
|
disease |
BEFREE |
Using a BMPR2 transgenic murine model of PAH and two models of inducible diabetes mellitus, we explored the impact of hyperglycemia and/or hyperinsulinemia on development and severity of PH.
|
28704134 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Patients with pulmonary arterial hypertension (PAH) can harbor mutations in several genes, most commonly in BMPR2.
|
30134121 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
In this study, we examined whether rats with the Bmpr2 mutation were susceptible to developing more severe PAH.
|
30586714 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
Biomarker
|
disease |
BEFREE |
Treatment with BMPR2-BM-ELPC attenuated PAH as demonstrated by a reduction in right ventricular hypertrophy as well as right ventricular systolic and mean pulmonary arterial pressures.
|
30977250 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
Biomarker
|
disease |
BEFREE |
Arterial remodeling-a hallmark of many cardiovascular pathologies including pulmonary arterial hypertension (PAH)-is regulated by TGFβ1 (transforming growth factor-β1)-TGFβ receptors and the antagonistic, vasoprotective BMPR2 (bone morphogenetic protein receptor 2)-PPARγ (peroxisome proliferator-activated receptor-γ) axis.
|
31023188 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
Biomarker
|
disease |
BEFREE |
Here we show that the receptor BMP type 2 (BMPR2) serves as a central gatekeeper of this balance, highlighted by its deregulation in diseases such as pulmonary arterial hypertension (PAH).
|
31826007 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Loss of function mutations in the type II BMP receptor BMPR2 are the leading cause of pulmonary arterial hypertension (PAH), a rare disease of vascular occlusion that leads to high blood pressure in the pulmonary arteries.
|
31797984 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
BMPR2 mutations and endothelial dysfunction in pulmonary arterial hypertension (2017 Grover Conference Series).
|
29521190 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
Biomarker
|
disease |
BEFREE |
Thus, we conjectured that miR-191, in ASCs and ASCs-Exos, plays an important role in PAH via regulation of BMPR2.
|
31119161 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
Biomarker
|
disease |
BEFREE |
R899X<sup>+/-</sup> BMPR2 transgenic mice fed a Western diet for six weeks were given daily injections of IL-1ß prior to assessment for PAH and tissue collection.
|
28828907 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Therefore, the subject was considered to have had heritable PAH due to a BMPR2 gene mutation.
|
31302445 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
HPAEC made dysfunctional by siRNA-mediated BMPR2 depletion showed downregulation of 18 and upregulation of 19 P2 receptor Ca<sup>2+</sup> signalosome genes including PLCD4, which was found to be upregulated in iPSC-EC from BMPR2-mutant patients with pulmonary arterial hypertension.
|
31396838 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
Heritable pulmonary arterial hypertension (PAH) is one such disorder characterised by rare mutations mostly occurring in the bone morphogenetic protein receptor type 2 (<i>BMPR2</i>) gene and a wide heterogeneity of penetrance modifier mechanisms.
|
30894412 |
2019 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
AlteredExpression
|
disease |
BEFREE |
Reduced bone morphogenetic protein receptor 2 (BMPR2) expression in patients with PAH impairs pulmonary arterial endothelial cells (PAECs) function.
|
29374556 |
2018 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
GeneticVariation
|
disease |
BEFREE |
GCN2 expression was quantified by Western blotting in 24 PVOD patients, 44 patients with pulmonary arterial hypertension (PAH; 23 bone morphogenetic protein receptor type II [BMPR2] mutation carriers, 21 non-carriers), and 3 experimental pulmonary hypertension models.
|
29108819 |
2018 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
Biomarker
|
disease |
BEFREE |
Full elucidation of BMPR2-mediated pathogenic mechanisms in PAH requires persistent efforts to achieve precision or individualized medicine as a therapeutic strategy for PAH.
|
29023671 |
2018 |
Idiopathic pulmonary arterial hypertension
|
0.700 |
Biomarker
|
disease |
BEFREE |
Baicalein attenuated MCT-induced PAH in rats by inhibiting EndoMT partially via the NF-κB-BMPR2 pathway.
|
29969608 |
2018 |