Fibulin 5 mediates pulmonary artery smooth muscle cell dysfunction and vascular remodelling in congenital heart disease associated pulmonary arterial hypertension via TGF-β1/PI3K/AKT signalling
Overall, our data provide new insights into the influence of fibulin 5 on the modification of hPASMC dysfunction and pulmonary artery remodelling in shunt-associated PAH.
Authors
- Yuling Qian (ORCID: https://orcid.org/0000-0001-5807-4123)
- En Qiao
- Yicheng Yang (ORCID: https://orcid.org/0000-0003-2884-2341)
- HE Zhi-yu (ORCID: https://orcid.org/0000-0002-6105-8834)
- Jingjing Zhou (ORCID: https://orcid.org/0009-0009-6790-9028)
Institutions
- Chinese Academy of Medical Sciences & Peking Union Medical College (CN)
- Sir Run Run Shaw Hospital (CN)
- First Affiliated Hospital of Guangzhou Medical University (CN)
- Beijing Anzhen Hospital (CN)
- First Affiliated Hospital Zhejiang University (CN)
- Guangzhou Medical University (CN)
Publication Details
- Journal
- Respiratory Research
- Published
- 2025-09-29
- DOI
- https://doi.org/10.1186/s12931-025-03361-z
- Primary Topic
- Pulmonary Hypertension Research and Treatments
- Type
- article
- Field-Weighted Citation Impact
- 0.00