CXCL12/CXCR4 pathway as a novel therapeutic target for RNF213-associated pulmonary arterial hypertension
Takahiro Hiraide, Noboru Tsuda, Mizuki Momoi, Yoshiki Shinya, Motoaki Sano, Keiichi Fukuda, Junji Shibahara, Junko Kuramoto, Yae Kanai, Kenjiro Kosaki, Yoji Hakamata, Masaharu Kataoka · 2024
Read CXCL12/CXCR4 pathway as a novel therapeutic target for RNF213-associated pulmonary arterial hypertension by Takahiro Hiraide, Noboru Tsuda, Mizuki Momoi, Yoshiki Shinya, Motoaki Sano, Keiichi Fukuda, Junji Shibahara, Junko Kuramoto, Yae Kanai, Kenjiro Kosaki, Yoji Hakamata, Masaharu Kataoka (2024) in the Tower of Babel, a free online library of public-domain works preserved in clean, searchable text. No sign-up. The full text is served as a plain-text file at a predictable URL, where text is present: https://antarctic-labs.com/tower-of-babel/pmc-11535198.txt.
Description
Takahiro Hiraide and his ten co-authors, publishing in 2024, trace how a gene variant drives a severe form of high blood pressure in the lungs. The variant, in a gene called RNF213, marks patients who respond poorly to treatment. Its mechanism was unknown. The team engineered mice carrying the mouse version of the variant and exposed them to low oxygen for three months.
The engineered mice developed the disease signs: higher lung artery pressure, a thickened right heart, and thicker vessel walls. A signaling molecule called CXCL12 ran high in their lungs. Blocking its receptor eased the disease. The same signal was active in lung tissue from severely ill patients carrying the variant. The authors conclude the variant causes this hypertension through that pathway. They propose blocking it as a treatment strategy for this genetic form of the disease.
Catalog record
| CREATOR | Takahiro Hiraide, Noboru Tsuda, Mizuki Momoi, Yoshiki Shinya, Motoaki Sano, Keiichi Fukuda, Junji Shibahara, Junko Kuramoto, Yae Kanai, Kenjiro Kosaki, Yoji Hakamata, Masaharu Kataoka |
|---|---|
| YEAR | 2024 |
| COLLECTION | PAPERS |
| FORMAT | TXT |
| FILE SIZE | 29 KB |
| VERSION | 1.0 |
| LICENSE | CC BY 4.0 |
| RIGHTS | CC BY |
| SOURCE | Europe PMC |