4.7 Review

Islet-intrinsic effects of CFTR mutation

期刊

DIABETOLOGIA
卷 59, 期 7, 页码 1350-1355

出版社

SPRINGER
DOI: 10.1007/s00125-016-3936-1

关键词

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

资金

  1. Cystic Fibrosis Trust
  2. Diabetes and Wellness Foundation
  3. Wellcome Trust
  4. North Staffordshire Medical Institute
  5. Cystic Fibrosis Trust [RS32, SRC007] Funding Source: researchfish

向作者/读者索取更多资源

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 a role 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 more complicated 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.

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