title: The impact of Cystic Fibrosis Transmembrane Regulator Disruption on cardiac function and stress response.
abstract: BACKGROUND
  Altered cardiac function has been observed in cystic fibrosis transmembrane regulator (CFTR) knockout mice. However, whether this alteration is a direct effect of CFTR disruption in the heart, or is secondary due to systemic loss of CFTR, remains to be elucidated.
  
  
  METHODS
  Cardiac function of mice with muscle-specific or global knockout of CFTR was evaluated at baseline and under β-stimulation by MRI in vivo. Myocyte contractility and Ca2+ transients were measured in vitro.
  
  
  RESULTS
  Both CFTR knockout models showed increased twist and torsion at baseline. Response to β-stimulation was unaltered in muscle-specific CFTR knockout mice and was slightly decreased in global CFTR knockout mice. Aortic diameter was also decreased in both mouse models. No difference was observed in myocyte contractility and Ca2+ transients.
  
  
  CONCLUSIONS
  CFTR disruption leads to increased myocardial contractility at baseline, which may trigger untoward myocardial remodeling in CF patients that is independent of lung diseases.
authors.affiliation: Department of Biomedical Engineering, Case Western Reserve University, Cleveland, OH, USA; Case Center for Imaging Research, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Kai
authors.initials: K
authors.lastname: Jiang
authors.affiliation: Department of Biomedical Engineering, Case Western Reserve University, Cleveland, OH, USA; Case Center for Imaging Research, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Sen
authors.initials: S
authors.lastname: Jiao
authors.affiliation: Department of Genetics and Genome Sciences, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Megan
authors.initials: M
authors.lastname: Vitko
authors.affiliation: Department of Genetics and Genome Sciences, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Rebecca
authors.initials: R
authors.lastname: Darrah
authors.affiliation: Department of Biomedical Engineering, Case Western Reserve University, Cleveland, OH, USA; Department of Radiology, Case Western Reserve University, Cleveland, OH, USA; Department of Pediatrics, Case Western Reserve University, Cleveland, OH, USA; Case Center for Imaging Research, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Chris A
authors.initials: CA
authors.lastname: Flask
authors.affiliation: Department of Genetics and Genome Sciences, Case Western Reserve University, Cleveland, OH, USA; Department of Pediatrics, Case Western Reserve University, Cleveland, OH, USA.
authors.forename: Craig A
authors.initials: CA
authors.lastname: Hodges
authors.affiliation: Department of Biomedical Engineering, Case Western Reserve University, Cleveland, OH, USA; Department of Radiology, Case Western Reserve University, Cleveland, OH, USA; Department of Physiology and Biophysics, Case Western Reserve University, Cleveland, OH, USA; Case Center for Imaging Research, Case Western Reserve University, Cleveland, OH, USA. Electronic address: xin.yu@case.edu.
authors.forename: Xin
authors.initials: X
authors.lastname: Yu
mesh_terms: D058665:Adrenergic beta-1 Receptor Agonists / Q000494:pharmacology; D000818:Animals; D001011:Aorta / Q000473:pathology; D003550:Cystic Fibrosis / Q000378:metabolism / Q000503:physiopathology; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000378:metabolism*; D004280:Dobutamine / Q000494:pharmacology*; D006321:Heart / Q000187:drug effects / Q000503:physiopathology; D008279:Magnetic Resonance Imaging; D051379:Mice; D018181:Mice, Inbred CFTR; D018345:Mice, Knockout; D032383:Myocytes, Cardiac / Q000187:drug effects / Q000378:metabolism; D009929:Organ Size; D013268:Stimulation, Chemical; D020257:Ventricular Remodeling
chemical_list: D058665:Adrenergic beta-1 Receptor Agonists; D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D004280:Dobutamine
keywords: CFTR; cardiac myocyte; cystic fibrosis; left ventricular function; β-adrenergic stimulation