Transmembrane Protein 100 Expression on Endothelial Cells Vascularizing Thrombi in Chronic Thromboembolic Pulmonary Hypertension Modulates TGFβ1−ALK1 Signaling During Angiogenesis

Pulmonary Circulation 2026 Open access

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Authors: Magdalena L. Bochenek, Iman Ghasemi, Christoph B. Wiedenroth, Olympia Bikou, Ioannis Karampinis, Eric Dominic Roessner, Lukas Hobohm, Stefan Guth, Philipp C. Lurz, Stavros Konstantinides, Katrin Schäfer
DOI: 10.1002/pul2.70253
Cited by: 0
References: 20

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.

Fields of Study & Subjects

Pulmonary Hypertension Research and Treatments TGF-β signaling in diseases Vascular Anomalies and Treatments

Publication details

Journal / source
Pulmonary Circulation
Volume
16
Issue
1
Pages
e70253–e70253
Publication date
1 January 2026
Language
EN