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Targeting Soluble TGF-β Factors

JACC Basic to Translational Science, vol. 9, pp. 1360–1374

Abstract

Pulmonary arterial hypertension (PAH) is a rare progressive disease characterized by pulmonary artery vascular remodeling, increased vascular resistance, and subsequent right ventricular hypertrophy and right heart failure. It is triggered by disrupted transforming growth factor (TGF)-β signaling, including loss-of-function mutations in the bone morphogenetic protein (BMP) receptor 2. Emerging treatments aim to inhibit elevated TGF-β levels or enhance diminished endothelial BMP signaling. This review aims to summarize the role of the TGF-β superfamily in the pathobiology of PAH and recent discoveries highlighting altered expression of TGF-β-related soluble factors in PAH patients that can serve as potential biomarkers and drug targets. The discussion focuses on how these altered factors can guide treatment decisions and monitor therapeutic responses, facilitating personalized patient care through the integration of diagnostics and therapy, that is, precision medicine. This approach tailors treatment strategies to individual patients based on their unique disease characteristics.

Authors 5

  1. Leiden University Medical Center

    Affiliation as printed

    Department of Cell and Chemical Biology, Leiden University Medical Center (LUMC), Leiden, the Netherlands

  2. Leiden University Medical Center

    Affiliation as printed

    Department of Cell and Chemical Biology, Leiden University Medical Center (LUMC), Leiden, the Netherlands

  3. Amsterdam University Medical Centers · Amsterdam Cardiovascular Sciences · Vrije Universiteit Amsterdam · Amsterdam UMC Location Vrije Universiteit Amsterdam

    Affiliation as printed

    Amsterdam Cardiovascular Sciences, Pulmonary Hypertension and Thrombosis, Amsterdam, the Netherlands

    Department of Pulmonary Medicine, Amsterdam University Medical Center, Vrije Universiteit, Amsterdam, the Netherlands

  4. Consejo Superior de Investigaciones Científicas · Leiden University Medical Center · Nanomaterials and Nanotechnology Research Center · Instituto de Investigación Sanitaria del Principado de Asturias

    Affiliation as printed

    Department of Cell and Chemical Biology, Leiden University Medical Center (LUMC), Leiden, the Netherlands

    Nanomaterials and Nanotechnology Research Center (CINN), Spanish Research National Council (CSIC) Health Research Institute of Asturias (ISPA), Oviedo, Spain

  5. Leiden University Medical Center

    Affiliation as printed

    Department of Cell and Chemical Biology, Leiden University Medical Center (LUMC), Leiden, the Netherlands

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References 133