Structural and transcriptomic alterations underlying the progression of aortic dissection in Fbn1G234D/G234D mice
smooth muscle cells (SMCs). Consistently, increased FN1 expression was observed in human non-heritable AD samples. Furthermore, enhanced expression of fibronectin receptors and activation of focal adhesion kinase signaling suggested augmented extracellular matrix-SMC interactions during disease progression. These findings indicate that AD progression involves coordinated medial structural failure, adventitial remodeling, and fibronectin-associated SMC dysfunction.
Authors
- Hiroko Matsunaga (ORCID: https://orcid.org/0000-0002-1725-3333)
- Haruko Takeyama (ORCID: https://orcid.org/0000-0002-2058-8185)
- Lynn Y. Sakai (ORCID: https://orcid.org/0000-0003-0054-1539)
- Chiho Tokunaga (ORCID: https://orcid.org/0000-0002-1349-4148)
- Sachiko Kanki (ORCID: https://orcid.org/0000-0003-4530-6006)
- Patrick Sips (ORCID: https://orcid.org/0000-0001-9241-5980)
- Violette Deleeuw (ORCID: https://orcid.org/0000-0001-8733-1854)
- Shigeki Koizumi (ORCID: https://orcid.org/0000-0003-3778-3371)
- Kaori Sugiyama (ORCID: https://orcid.org/0000-0002-4027-0705)
- Eri Motoyama
- Haruka Ozaki
- Md Al Amin Sheikh
- Yuji Hiramatsu
- Kenichi Kimura
- Keiichi Asano
- Julie De Backer
- Hiromi Yanagisawa
Institutions
- University of Tsukuba (JP)
- Waseda University (JP)
- Oregon Health & Science University (US)
- Ghent University Hospital (BE)
- Ghent University (BE)
- RIKEN Center for Biosystems Dynamics Research (JP)
- National Defense Medical College (JP)
- Osaka University of Pharmaceutical Sciences (JP)
- The University of Osaka (JP)
Publication Details
- Journal
- Scientific Reports
- Published
- 2026-04-27
- DOI
- https://doi.org/10.1038/s41598-026-50847-x
- Primary Topic
- Congenital heart defects research
- Type
- article
- Field-Weighted Citation Impact
- 0.00
Funders
- Japan Agency for Medical Research and Development
- Marfan Foundation
- Diamond Light Source
- Paul Scherrer Institut
- University of Tsukuba
- Japan Society for the Promotion of Science