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

Westholm, Efraim LU ; Wendt, Anna LU and Eliasson, Lena LU orcid (2021) In Clinical Medicine Insights: Endocrinology and Diabetes 14. p.1-7
Abstract
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... (More)
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 (Less)
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author
; and
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Clinical Medicine Insights: Endocrinology and Diabetes
volume
14
pages
1 - 7
publisher
SAGE Publications
external identifiers
  • pmid:34345195
  • scopus:85110032547
ISSN
1179-5514
DOI
10.1177/11795514211031204
language
English
LU publication?
yes
id
83aa37d0-daa7-47ab-87fd-075b8e888436
date added to LUP
2022-01-24 13:33:27
date last changed
2022-04-27 07:17:25
@article{83aa37d0-daa7-47ab-87fd-075b8e888436,
  abstract     = {{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}},
  author       = {{Westholm, Efraim and Wendt, Anna and Eliasson, Lena}},
  issn         = {{1179-5514}},
  language     = {{eng}},
  pages        = {{1--7}},
  publisher    = {{SAGE Publications}},
  series       = {{Clinical Medicine Insights: Endocrinology and Diabetes}},
  title        = {{Islet Function in the Pathogenesis of Cystic Fibrosis-Related Diabetes Mellitus}},
  url          = {{http://dx.doi.org/10.1177/11795514211031204}},
  doi          = {{10.1177/11795514211031204}},
  volume       = {{14}},
  year         = {{2021}},
}