Cystic Fibrosis and the Nervous System

Chest. 2017 May;151(5):1147-1155. doi: 10.1016/j.chest.2016.11.009. Epub 2016 Nov 19.


Cystic fibrosis (CF) is a life-shortening autosomal recessive disorder caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR). CFTR is an anion channel that conducts bicarbonate and chloride across cell membranes. Although defective anion transport across epithelial cells is accepted as the basic defect in CF, many of the features observed in people with CF and organs affected by CF are modulated by the nervous system. This is of interest because CFTR expression has been reported in both the peripheral and central nervous systems, and it is well known that the transport of anions, such as chloride, greatly modulates neuronal excitability. Thus it is predicted that in CF, lack of CFTR in the nervous system affects neuronal function. Consistent with this prediction, several nervous system abnormalities and nervous system disorders have been described in people with CF and in animal models of CF. The goal of this special feature article is to highlight the expression and function of CFTR in the nervous system. Special emphasis is placed on nervous system abnormalities described in people with CF and in animal models of CF. Finally, features of CF that may be modulated by or attributed to faulty nervous system function are discussed.

Keywords: CFTR; cough; cystic fibrosis; glucose regulation; mucus secretion; nervous system.

Publication types

  • Review
  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Autonomic Nervous System / metabolism
  • Autonomic Nervous System / physiopathology
  • Central Nervous System / metabolism*
  • Central Nervous System / physiopathology
  • Cough
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / genetics*
  • Cystic Fibrosis / physiopathology
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Cystic Fibrosis Transmembrane Conductance Regulator / metabolism*
  • Disease Models, Animal
  • Exocrine Pancreatic Insufficiency / etiology
  • Exocrine Pancreatic Insufficiency / metabolism
  • Exocrine Pancreatic Insufficiency / physiopathology
  • Gastrointestinal Motility
  • Gastrointestinal Tract / innervation
  • Heart / innervation
  • Humans
  • Intestinal Obstruction / etiology
  • Intestinal Obstruction / metabolism
  • Intestinal Obstruction / physiopathology
  • Malabsorption Syndromes / etiology
  • Malabsorption Syndromes / metabolism
  • Malabsorption Syndromes / physiopathology
  • Muscle Contraction
  • Muscle, Smooth / innervation
  • Pancreas / innervation
  • Peripheral Nervous System / metabolism*
  • Peripheral Nervous System / physiopathology
  • Respiratory System / innervation
  • Sweating


  • Cystic Fibrosis Transmembrane Conductance Regulator