Publications by authors named "F Perros"
Article Synopsis
- Vitamin D deficiency in pulmonary arterial hypertension (PAH) patients is linked to a poorer prognosis, and this study investigates the role of the vitamin D receptor (VDR) in the pulmonary vasculature.
- VDR is found in pulmonary artery smooth muscle cells (PASMC), and its expression is lower in PAH patients compared to non-hypertensive controls, but can be increased by calcitriol treatment.
- Calcitriol not only raises VDR expression but also has an antiproliferative effect on PASMC, which involves the modulation of specific genes like survivin and BMP signaling, suggesting potential therapeutic implications for PAH.
Background: Integrative multiomics can elucidate pulmonary arterial hypertension (PAH) pathobiology, but procuring human PAH lung samples is rare.
Methods: We leveraged transcriptomic profiling and deep phenotyping of the largest multicenter PAH lung biobank to date (96 disease and 52 control) by integration with clinicopathologic data, genome-wide association studies, Bayesian regulatory networks, single-cell transcriptomics, and pharmacotranscriptomics.
Results: We identified 2 potentially protective gene network modules associated with vascular cells, and we validated , coding for asporin, as a key hub gene that is upregulated as a compensatory response to counteract PAH.
Pulmonary arterial hypertension (PAH) is a chronic disorder characterized by excessive pulmonary vascular remodeling, leading to elevated pulmonary vascular resistance and right ventricle (RV) overload and failure. MicroRNA-146a (miR-146a) promotes vascular smooth muscle cell proliferation and vascular neointimal hyperplasia, both hallmarks of PAH. This study aimed to investigate the effects of miR-146a through pharmacological or genetic inhibition on experimental PAH and RV pressure overload animal models.
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- Pathogenic variants in a specific gene significantly increase the risk of developing heritable pulmonary arterial hypertension, and examining unaffected carriers through advanced screening methods can help in early diagnosis and understanding of the condition.
- A study compared 28 unaffected carriers to 21 healthy controls using various cardiac imaging and exercise tests; findings revealed carriers had smaller heart volumes but higher afterload, indicating potential early signs of the disease.
- Over a 4-year follow-up, two unaffected carriers went on to develop pulmonary arterial hypertension, despite showing normal cardiac indices at the time of diagnosis, highlighting the importance of monitoring these individuals for early intervention.