Targeting soluble TGF-β factors: advances in precision therapy for pulmonary arterial hypertension

Becher, C.; Wits, M.; Man, F.S. de; Sanchez Duffhues, G.; Goumans, M.J.

doi:doi:10.1016/j.jacbts.2024.04.005

Persistent URL of this record https://hdl.handle.net/1887/4291990

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Medicine / Leiden University Medical Centre (LUMC)

Becher, C.; Wits, M.; Man, F.S. de; Sanchez Duffhues, G.; Goumans, M.J. (2024)

Targeting soluble TGF-β factors: advances in precision therapy for pulmonary arterial hypertension

Article / Letter to editor

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 Show morePulmonary 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.Show less

BMPRII

endothelial

miRNAs

small extracellular vesicles

smooth muscle cell