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Generation of Human Nasal Epithelial Cell Spheroids for Individualized Cystic Fibrosis Transmembrane Conductance Regulator Study
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The impact of Cystic Fibrosis Transmembrane Regulator Disruption on cardiac function and stress response

Kai Jiang1, Sen Jiao1, Megan Vitko2

  • 1Department of Biomedical Engineering, Case Western Reserve University, Cleveland, OH, USA; Case Center for Imaging Research, Case Western Reserve University, Cleveland, OH, USA.

Journal of Cystic Fibrosis : Official Journal of the European Cystic Fibrosis Society
|June 30, 2015
PubMed
Abstract

Insights

Cystic fibrosis transmembrane regulator (CFTR) disruption in mice causes increased baseline myocardial contractility. This cardiac remodeling may occur independently of lung disease in cystic fibrosis patients.

Area of Science:

  • Cardiology
  • Genetics
  • Molecular Biology

Background:

  • Altered cardiac function is noted in cystic fibrosis transmembrane regulator (CFTR) knockout mice.
  • The direct cardiac impact of CFTR disruption versus systemic effects is unclear.

Purpose of the Study:

  • To investigate whether CFTR disruption directly impacts cardiac function.
  • To differentiate between direct cardiac effects and systemic consequences of CFTR loss.

Main Methods:

  • Cardiac function assessed via MRI in muscle-specific and global CFTR knockout mice.
  • In vitro myocyte contractility and Ca2+ transients were measured.

Main Results:

  • Both CFTR knockout models exhibited increased baseline cardiac twist and torsion.
  • Myocyte contractility and Ca2+ transients were not significantly different.
  • Aortic diameter was reduced in both knockout models.

Conclusions:

  • CFTR disruption directly increases baseline myocardial contractility.
  • This heightened contractility may promote cardiac remodeling in cystic fibrosis patients, irrespective of lung disease.
Keywords:
CFTRcardiac myocytecystic fibrosisleft ventricular functionβ-adrenergic stimulation

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