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Positive correlation between the dysregulation of transforming growth factor-β1 and aneurysmal pathological changes

Koung Li Kim1, Jeong Hoon Yang, Sun-Hwa Song

  • 1Department of Medicine, Samsung Medical Center, Samsung Biomedical Research Institute, Sungkyunkwan University School of Medicine, Seoul, Korea.

Circulation Journal : Official Journal of the Japanese Circulation Society
|January 8, 2013
PubMed
Abstract

Insights

Transforming growth factor-β1 (TGF-β1) dysregulation is strongly linked to aortic disease in Marfan syndrome (MFS) patients. Elevated TGF-β1 levels may serve as a biomarker for MFS aortic aneurysm progression.

Area of Science:

  • Cardiovascular Biology
  • Genetics
  • Pathology

Background:

  • Marfan syndrome (MFS) is a genetic disorder affecting connective tissue, often leading to aortic aneurysms.
  • While TGF-β signaling is implicated in MFS animal models, its role in human MFS aortic pathogenesis remains unclear.

Purpose of the Study:

  • To investigate the correlation between transforming growth factor-β1 (TGF-β1) dysregulation and cystic medial degeneration in MFS patients' aortic tissues.
  • To determine if TGF-β1 dysregulation is associated with pathogenic features of aortic aneurysms in humans with MFS.

Main Methods:

  • Histopathological analysis of aortic tissues from MFS patients and normal individuals.
  • Assessment of TGF-β1 expression and signaling activation (Smad2 phosphorylation) in aortic tissues and plasma.
  • Correlation analysis between TGF-β1 levels, signaling, and histopathological findings.

Main Results:

  • MFS aortic tissues showed significant cystic medial degeneration and apoptosis compared to controls.
  • Increased TGF-β1 expression and Smad2 phosphorylation were observed in MFS aortic tissues and plasma.
  • A positive correlation existed between TGF-β1 levels, signaling activation, and the severity of aortic degeneration in MFS patients.

Conclusions:

  • A strong association between TGF-β1 dysregulation and aortic pathogenesis in human MFS patients was identified.
  • Plasma TGF-β1 concentration may serve as a potential biomarker for monitoring aortic aneurysm progression in MFS.

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