Islet-intrinsic effects of CFTR mutation

Fiona N. Manderson Koivula, Neville H. McClenaghan, Alan G. S. Harper, Catriona Kelly

Research output: Contribution to journalArticlepeer-review

38 Citations (Scopus)


Cystic fibrosis-related diabetes (CFRD) is the most significant extra-pulmonary comorbidity in cystic fibrosis (CF) patients, and accelerates lung decline. In addition to the traditional view that CFRD is a consequence of fibrotic destruction of the pancreas as a whole, emerging evidence may implicate arole for cystic fibrosis transmembrane-conductance regulator (CFTR) in the regulation of insulin secretion from the pancreatic islet. Impaired first-phase insulin responses and glucose homeostasis have also been reported in CF patients. CFTR expression in both human and mouse beta cells has been confirmed, and recent studies have shown differences in endocrine pancreatic morphology from birth in CF. Recent experimental evidence suggests that functional CFTR channels are required for insulin exocytosis and the regulation of membrane potential in the pancreatic beta cell, which may account for the impairments in insulin secretion observed in many CF patients.These novel insights suggest that the pathogenesis of CFRD is morecomplicated than originally thought, with implications for diabetes treatment and screening in the CF population. This review summarises recent emerging evidence in support of a primary role for endocrine pancreatic dysfunction in the development of CFRD.
Original languageEnglish
Pages (from-to)1350-1355
Early online date31 Mar 2016
Publication statusPublished online - 31 Mar 2016


  • Beta cells
  • CFTR
  • Cystic fibrosis
  • Diabetes
  • Endocrine
  • Review


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