Researchers found that a transforming growth factor-beta 1 (TGF-β1)-inducible protein called transgelin exacerbated pulmonary arterial smooth muscle cell (PASMC) dysfunction in a rat model, leading to pulmonary arterial hypertension (PAH), particularly in congenital heart disease (CHD)-related PAH.

The study, published in ESC Heart Failure, found overexpression of transgelin in both PAH rats and in lung tissues from patients with CHD-PAH, suggesting that the protein may therefore represent a potential new treatment target in CHD-PAH.

“Our group demonstrated that transgelin was markedly elevated in irreversible CHD-PAH patients compared with reversible CHD-PAH and normal control groups, and its expression was positively correlated with pathological grading of pulmonary arterioles, which implied that transgelin may be involved in the progression of CHD-PAH,” the authors wrote.

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“In this study, we aimed to probe the molecular mechanism and biological function of transgelin in the pathogenesis of CHD-PAH.”

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To investigate transgelin expression in lung tissues in CHD-PAH, the research team enrolled 19 patients with CHD-PAH at a single center in China and obtained lung tissue samples from 12 of those who underwent surgery. In addition, the role of trangelin in pulmonary vascular remodeling and PAH development was explored in lung tissue samples from 66 adult male Sprague-Dawley rats with monocrotaline plus aortocaval-induced PAH.

The results showed that transgelin was significantly overexpressed in both the pulmonary arterioles of the CHD-PAH patients and the PAH rats, and transgelin levels correlated with the pathological grading of the patients’ arteries with PAH.

Furthermore, TGF-β1 was found to promote transgelin expression as PAH progressed, thereby exacerbating PASMC dysfunction. The authors suggest that these results imply that transgelin could be a possible treatment target for pulmonary vascular remodeling in CHD-PAH.


Zhou, J, Yang J, Li L, et al. Transgelin exacerbates pulmonary artery smooth muscle cell dysfunction in shunt-related pulmonary arterial hypertension. ESC Heart Failure. Published online July 15, 2022. doi:10.1002/ehf2.14080