Islet Function in the Pathogenesis of Cystic Fibrosis-Related Diabetes Mellitus

Autor: Efraim Westholm, Anna Wendt, Lena Eliasson
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Zdroj: Clinical Medicine Insights: Endocrinology and Diabetes, Vol 14 (2021)
Druh dokumentu: article
ISSN: 1179-5514
11795514
DOI: 10.1177/11795514211031204
Popis: Cystic fibrosis-related diabetes mellitus (CFRD) is the most common non-pulmonary co-morbidity in cystic fibrosis (CF). CF is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene ( CFTR ), which leads to aberrant luminal fluid secretions in organs such as the lungs and pancreas. How dysfunctional CFTR leads to CFRD is still under debate. Both intrinsic effects of dysfunctional CFTR in hormone secreting cells of the islets and effects of exocrine damage have been proposed. In the current review, we discuss these non-mutually exclusive hypotheses with a special focus on how dysfunctional CFTR in endocrine cells may contribute to an altered glucose homeostasis. We outline the proposed role of CFTR in the molecular pathways of β-cell insulin secretion and α-cell glucagon secretion, and touch upon the importance of the exocrine pancreas and intra-pancreatic crosstalk for proper islet function.
Databáze: Directory of Open Access Journals