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Updated: Feb 4, 2026

Chronic Thromboembolic Pulmonary Hypertension and Assessment of Right Ventricular Function in the Piglet
Published on: November 4, 2015
Transmembrane Protein 100 Expression on Endothelial Cells Vascularizing Thrombi in Chronic Thromboembolic Pulmonary
Magdalena L Bochenek1,2,3, Iman Ghasemi1,3, Christoph B Wiedenroth4
1Department of Cardiology, Cardiology I University Medical Center Mainz Mainz Germany.
Abstract:
Endothelial cells within chronic pulmonary artery thrombi in CTEPH overexpress transmembrane protein 100 (TMEM100), an activin A receptor-like kinase 1 (ACVRL1 or ALK1) signaling-dependent gene, and TGFβ1 upregulated TMEM100 transcription in healthy lung ECs. TMEM100 permitted the TGFβ1-induced increase of ALK1, while repressing ALK5, and preventing ALK1-TMEM100 signaling impaired angiogenesis ex vivo. Our data indicate that TGFβ1-ALK1-TMEM100 signaling is active during CTEPH thrombus revascularization.
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