Deficiency of Smooth Muscle Adar1 Exacerbates Vascular Remodeling and Pulmonary Hypertension
Our study describes a fundamental molecular mechanism underlying the progression of PH. We highlight the detrimental role of innate immune responses, where smooth muscle cell and context-specific RNA editing, along with the sensing of dsRNA, mediate disease progression and excessive vascular remodeling. This finding suggests that targeting PKR could be the new therapeutic strategy for treating pulmonary arterial hypertension.
Authors
- Yan Li (ORCID: https://orcid.org/0000-0003-4504-558X)
- Yunhye Kim (ORCID: https://orcid.org/0009-0002-1804-571X)
- Chanil Valasarajan (ORCID: https://orcid.org/0000-0002-0969-9044)
- Theodore Avolio
- Yuan Hao (ORCID: https://orcid.org/0000-0003-2322-6806)
- Chen-Shan Chen Woodcock (ORCID: https://orcid.org/0000-0003-4291-1788)
- Thomas Bertero (ORCID: https://orcid.org/0000-0002-4801-9902)
- Stephen Y. Chan (ORCID: https://orcid.org/0000-0002-9520-7527)
- Elena A. Goncharova (ORCID: https://orcid.org/0000-0001-7118-6752)
- Clemens Ruppert (ORCID: https://orcid.org/0000-0002-0938-2469)
- Joseph C. Wu (ORCID: https://orcid.org/0000-0002-6068-8041)
- Soni Savai Pullamsetti (ORCID: https://orcid.org/0000-0003-0440-8831)
- Benjamin A. Raby (ORCID: https://orcid.org/0000-0003-2206-5748)
- Rajkumar Savai (ORCID: https://orcid.org/0000-0003-1538-2091)
- Carsten Kuenne (ORCID: https://orcid.org/0000-0001-8013-5906)
- Yi‐Yin Tai (ORCID: https://orcid.org/0000-0003-3674-0783)
- Jin Billy Li (ORCID: https://orcid.org/0000-0003-0713-1399)
- A. A. Roger Thompson (ORCID: https://orcid.org/0000-0002-0717-4551)
- Tatiana V. Kudryashova (ORCID: https://orcid.org/0000-0002-2843-5554)
- Giovanni Maroli (ORCID: https://orcid.org/0000-0001-8245-5348)
- Mario Looso (ORCID: https://orcid.org/0000-0003-1495-9530)
- H. Turton (ORCID: https://orcid.org/0000-0003-1178-4835)
- Valerié Schumacher (ORCID: https://orcid.org/0000-0001-8670-0486)
- Yu Liu (ORCID: https://orcid.org/0000-0001-7110-127X)
- Ying Tang (ORCID: https://orcid.org/0000-0002-3569-6189)
- Ke Yuan (ORCID: https://orcid.org/0000-0001-8085-5452)
- Hyunbum Kim (ORCID: https://orcid.org/0000-0002-7193-0278)
- Qin Li (ORCID: https://orcid.org/0000-0002-8948-2717)
- Tim Klouda
Institutions
- Boston Children's Hospital (US)
- Centre National de la Recherche Scientifique (FR)
- Beth Israel Deaconess Medical Center (US)
- Justus-Liebig-Universität Gießen (DE)
- Université Côte d'Azur (FR)
- University of California System (US)
- National Jewish Health (US)
- German Center for Lung Research (DE)
- Universities of Giessen and Marburg Lung Center (DE)
- University of Pittsburgh Medical Center (US)
- Cardio-Pulmonary Institute (DE)
- Institut de Pharmacologie Moléculaire et Cellulaire (FR)
- University of Pennsylvania (US)
- University of Sheffield (GB)
- University of Colorado Denver (US)
- Stanford University (US)
Publication Details
- Journal
- Circulation Research
- Published
- 2025-10-22
- DOI
- https://doi.org/10.1161/circresaha.125.326235
- Citations
- 2
- Primary Topic
- RNA regulation and disease
- Type
- article
- Field-Weighted Citation Impact
- 1.61