GEOapplication/xml Mus musculusHomo sapiensExpression profiling by high throughput sequencing role of CFTR in islet functionCystic fibrosis (CF)-related diabetes (CFRD) is an increasingly common and devastating comorbidity of CF, affecting ~35% of adults with CF. However, the underlying causes of CFRD are unclear. Here, we examined cystic fibrosis transmembrane conductance regulator (CFTR) islet expression and whether the CFTR participates in islet endocrine cell function using murine models of b cell CFTR deletion, and normal and CF human pancreas and islets. Specific deletion of CFTR from murine b cells did not affect b cell function. In human islets, CFTR mRNA was minimally expressed, and CFTR protein/electrical activity was not detected. Isolated CF/CFRD islets demonstrated appropriate insulin and glucagon secretion with few changes in key islet-regulatory transcripts. Furthermore, ~65% of b cell area was lost in CF donors, compounded by pancreatic remodeling and immune infiltration of the islet. These results indicate that CFRD is not caused by intrinsic islet dysfunction from CFTR mutation, but rather, by b cell loss and intra-islet inflammation in the setting of a complex pleiotropic disease2018/04/25GSE110935GSM3019105GSM3019106GSM3019107GSM3019101GSM3019102GSM3019103GSM3019104GSM3019097GSM3019086GSM3019087GSM3019098GSM3019099GSM3019088GSM3019100GSM3019089GSM3019093GSM3019094GSM3019095GSM3019096GSM3019090GSM3019091GSM30190921679117021SRP133211110935 Mus musculusHomo sapiens[29669939]