Heterogeneous expression of CFTR in insulin-secreting β-cells of the normal human islet.

ABSTRACT: Cystic fibrosis (CF) is due to mutations in the CF-transmembrane conductance regulator (CFTR) and CF-related diabetes (CFRD) is its most common co-morbidity, affecting ~50% of all CF patients, significantly influencing pulmonary function and longevity. Yet, the complex pathogenesis of CFRD remains unclear. Two non-mutually exclusive underlying mechanisms have been proposed in CFRD: i) damage of the endocrine cells secondary to the severe exocrine pancreatic pathology and ii) intrinsic β-cell impairment of the secretory response in combination with other factors. The later has proven difficult to determine due to low expression of CFTR in β-cells, which results in the general perception that this Cl-channel does not participate in the modulation of insulin secretion or the development of CF

SUBMITTER: Di Fulvio M 

PROVIDER: S-EPMC7710116 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature