The proprotein convertase FURIN is a novel aneurysm predisposition gene impairing TGF-β signalling
2024

FURIN: A New Gene Linked to Aortic Aneurysms

Sample size: 781 publication 10 minutes Evidence: moderate

Author Information

Author(s): Zongsheng He, Arne S. IJpma, Dianne Vreeken, Daphne Heijsman, Karen Rosier, Hence J.M. Verhagen, Jorg L. de Bruin, Hennie T. Brüggenwirth, Jolien W. Roos-Hesselink, Jos A. Bekkers, Danny Huylebroeck, John W.M. Creemers, Danielle Majoor-Krakauer

Primary Institution: Erasmus MC University Medical Center

Hypothesis

Is FURIN a genetic predisposition gene for aortic aneurysms affecting TGF-β signaling?

Conclusion

FURIN is identified as a frequent genetic predisposition for various types of aortic aneurysms by impairing TGF-β signaling.

Supporting Evidence

  • FURIN variants were found in 3.7% of the aortic aneurysm patients studied.
  • 72% of patients with FURIN variants had multiple aneurysms.
  • Impaired TGF-β signaling was observed in patient-derived fibroblasts with FURIN variants.
  • FURIN variants were linked to decreased levels of collagen and fibrillin in aortic tissue.

Takeaway

Scientists found a gene called FURIN that can make people more likely to have problems with their aorta, which is a big blood vessel in the body.

Methodology

Whole-exome sequencing was performed on 781 aortic aneurysm patients to identify rare FURIN variants.

Limitations

The study population size limited the identification of factors contributing to the polygenic effect of FURIN variants.

Participant Demographics

The study included 781 unrelated aortic aneurysm patients, with a mean age of 63 years at diagnosis.

Statistical Information

P-Value

p<0.05

Statistical Significance

p<0.05

Digital Object Identifier (DOI)

10.1093/cvr/cvae078

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