The role of islet CFTR in the development of cystic fibrosis-related diabetes : A semi-systematic review
Engström, Matilda LU ; Westholm, Efraim LU ; Wendt, Anna LU and Eliasson, Lena LU
(2025)
In Journal of Cystic Fibrosis
24(4).
p.724-732
- Abstract
Background: Cystic fibrosis related diabetes (CFRD) is the most common comorbidity of cystic fibrosis (CF) still, its pathogenesis is poorly understood. Recent studies have suggested that although pancreatic insufficiency is an important explanation for CFRD development, inherent pancreatic islet cell dysfunction may play a role. This study aimed to systematically compile current data regarding the impact of pancreatic islet cell dysfunction on the development of CFRD. Methods: A systematic search was conducted in PubMed and Embase. The resulting articles were screened for relevant experimental design and outcomes. Articles underwent data extraction and quality assessment before compilation and analysis of the results. Results: A total... (More)
Background: Cystic fibrosis related diabetes (CFRD) is the most common comorbidity of cystic fibrosis (CF) still, its pathogenesis is poorly understood. Recent studies have suggested that although pancreatic insufficiency is an important explanation for CFRD development, inherent pancreatic islet cell dysfunction may play a role. This study aimed to systematically compile current data regarding the impact of pancreatic islet cell dysfunction on the development of CFRD. Methods: A systematic search was conducted in PubMed and Embase. The resulting articles were screened for relevant experimental design and outcomes. Articles underwent data extraction and quality assessment before compilation and analysis of the results. Results: A total of 268 articles were initially screened and 19 studies conducted between 2006–2022 were finally included in this review. Half of the studies in human tissue and most of the studies in animal tissue could detect CFTR in the islets. Similarly, half of the publications in human islets and most studies in animal islets detect decreased insulin secretion with inhibition/mutation of CFTR. Conclusions: The literature on the role of islet CFTR is contradictory. However, a pattern emerges where CFTR loss-of-function mutations have the potential to negatively affect islet cell function in a way that, together with previously described exocrine damage occurring in CF, could play a part in the development of CFRD.
(Less)
- author
- Engström, Matilda
LU
; Westholm, Efraim
LU
; Wendt, Anna
LU
and Eliasson, Lena
LU
- organization
- publishing date
- 2025-07
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- CFRD, CFTR, Diabetes, Glucagon, Insulin, Islet
- in
- Journal of Cystic Fibrosis
- volume
- 24
- issue
- 4
- pages
- 9 pages
- publisher
- Elsevier
- external identifiers
-
- pmid:40254519
- scopus:105002927106
- ISSN
- 1569-1993
- DOI
- 10.1016/j.jcf.2025.04.006
- language
- English
- LU publication?
- yes
- additional info
- Publisher Copyright: © 2025 The Author(s)
- id
- bdf4e6ae-4715-4154-b731-24db91ef7871
- date added to LUP
- 2026-06-11 11:18:48
- date last changed
- 2026-06-12 03:02:18
@article{bdf4e6ae-4715-4154-b731-24db91ef7871,
abstract = {{<p>Background: Cystic fibrosis related diabetes (CFRD) is the most common comorbidity of cystic fibrosis (CF) still, its pathogenesis is poorly understood. Recent studies have suggested that although pancreatic insufficiency is an important explanation for CFRD development, inherent pancreatic islet cell dysfunction may play a role. This study aimed to systematically compile current data regarding the impact of pancreatic islet cell dysfunction on the development of CFRD. Methods: A systematic search was conducted in PubMed and Embase. The resulting articles were screened for relevant experimental design and outcomes. Articles underwent data extraction and quality assessment before compilation and analysis of the results. Results: A total of 268 articles were initially screened and 19 studies conducted between 2006–2022 were finally included in this review. Half of the studies in human tissue and most of the studies in animal tissue could detect CFTR in the islets. Similarly, half of the publications in human islets and most studies in animal islets detect decreased insulin secretion with inhibition/mutation of CFTR. Conclusions: The literature on the role of islet CFTR is contradictory. However, a pattern emerges where CFTR loss-of-function mutations have the potential to negatively affect islet cell function in a way that, together with previously described exocrine damage occurring in CF, could play a part in the development of CFRD.</p>}},
author = {{Engström, Matilda and Westholm, Efraim and Wendt, Anna and Eliasson, Lena}},
issn = {{1569-1993}},
keywords = {{CFRD; CFTR; Diabetes; Glucagon; Insulin; Islet}},
language = {{eng}},
number = {{4}},
pages = {{724--732}},
publisher = {{Elsevier}},
series = {{Journal of Cystic Fibrosis}},
title = {{The role of islet CFTR in the development of cystic fibrosis-related diabetes : A semi-systematic review}},
url = {{http://dx.doi.org/10.1016/j.jcf.2025.04.006}},
doi = {{10.1016/j.jcf.2025.04.006}},
volume = {{24}},
year = {{2025}},
}