In Applications, flexiVent
Cystic Fibrosis (CF) is an inherited autosomal recessive disease
resulting from mutations in the CF Transmembrane conductance Regulator (CFTR) gene. CF patients progressively develop a
pronounced respiratory phenotype, as the absence of CFTR function in the lung is
associated with the thickening of secretions as well as the inability to
properly excrete or clear them. This leads
to bacteria accumulation and eventually bronchiectasis, allergic
bronchopulmonary aspergillosis, and even respiratory failure.

The identification of the CFTR gene in the late 1980s propelled
CF research1.  CFTR was
identified as a chloride channel and knockout mice were generated, but the early phenotyping studies lacked the sensitivity
required to quantify respiratory changes in these mice. It wasn’t until the
detailed respiratory mechanics of the flexiVent that the first respiratory CFTR
phenotype was reported2.  Various
preclinical models of CF are now available3-5 to study the pulmonary
manifestations of the CF disease and respiratory mechanics remain an important study
outcome in that field.


There is also mounting evidence that CFTR plays a direct role
in the airway smooth muscle6. Measuring the reactivity of isolated tracheal
rings or strips ex vivo, in tissue baths, allows for a functional assessment in
absence of external influences.  This
approach was taken to study the effect of CFTR function on human airway smooth
muscle and to confirm its role in bronchorelaxation6.

The emka & SCIREQ team will be attending the American Thoracic Society’s 2017 conference in Washington, DC! Come visit our booth #1731 and speak with our experienced team about our solutions for preclinical pediatric research.

1Identification of the cystic fibrosis gene: chromosome
walking and jumping – Rommens et al. Science 245: 1059, 1989.
2 The “Goldilocks Effect” in Cystic Fibrosis: Identification
of a lung phenotype in cftr knockout
and heterozygous mouse – Cohen et al. BMC Genetics., 5: 21, 2004.
3Air trapping and airflow obstruction in newborn cystic
fibrosis piglets – Adams et al. Am J Respir Crit Care Med 188: 1434, 2013.
4Lung phenotype of juvenile
and adult cystic fibrosis transmembrane conductance regulator knockout ferrets
– Sun et al. Am J Respir Cell Mol Biol 50: 502, 2014.
5Early pulmonary disease manifestations in cystic
fibrosis mice – Darrah et al. Journal of Cystic Fibrosis 15: 736, 2016.

6Bronchorelaxation of
the human bronchi by CFTR activators – Norez et al. ‎Pulm Pharmacol Ther 27: 38,

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