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An Investigation of Fibulin-2 in Hypertrophic Cardiomyopathy

Ayman M Ibrahim1,2, Mohamed Roshdy1, Sara Elshorbagy1

  • 1Aswan Heart Center, Aswan 200, Egypt.

International Journal of Molecular Sciences
|October 2, 2020
PubMed

Insights

This study reveals abnormal fibulin-2 expression in hypertrophic cardiomyopathy (HCM) patients. Elevated fibulin-2 in heart tissue and serum may contribute to this common inherited heart muscle disease.

Area of Science:

  • Cardiology
  • Molecular Biology
  • Genetics

Background:

  • Hypertrophic cardiomyopathy (HCM) is a prevalent inherited heart muscle disease affecting at least 1 in 500 individuals.
  • HCM is characterized by myocardial stiffness and fibrosis, linked to extracellular matrix (ECM) alterations.
  • The specific roles of many ECM components in HCM pathogenesis remain unclear.

Purpose of the Study:

  • To investigate the expression and role of fibulin-2, an ECM glycoprotein, in the myocardium of patients with HCM.
  • To compare fibulin-2 levels in HCM patients versus healthy controls.

Main Methods:

  • Immunohistochemical analysis of myocardial tissue from HCM patients and controls.
  • Enzyme-linked immunosorbent assay (ELISA) to quantify serum fibulin-2 levels.

Main Results:

  • Abnormal fibulin-2 expression was observed in the cytoplasm of myocardial cells and interstitial fibroblasts in HCM patients.
  • Serum fibulin-2 levels were significantly elevated in HCM patients compared to normal controls.

Conclusions:

  • Fibulin-2 is abnormally expressed in the myocardium and elevated in the serum of patients with hypertrophic cardiomyopathy.
  • These findings suggest a potential role for fibulin-2 in the pathophysiology of HCM.

Keywords:
cardiac remodelingextracellular matrixfibroblastfibrosishypertrophy

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