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Unravelling the developmental origins of cystic fibrosis-related diabetes

Alex Cho1,2, Maria Cristina Nostro3,4

  • 1McEwen Stem Cell Institute, University Health Network, Toronto, ON, Canada.

Diabetologia
|June 9, 2026
PubMed

Insights

Cystic fibrosis (CF)-related diabetes (CFRD) may stem from impaired pancreatic development caused by CF transmembrane conductance regulator (CFTR) gene mutations. Understanding CFTR's role in pancreatic organogenesis is key to improving CFRD care.

Area of Science:

  • Endocrinology
  • Genetics
  • Developmental Biology

Background:

  • Cystic fibrosis (CF)-related diabetes (CFRD) is a significant complication in cystic fibrosis (CF) patients.
  • Current understanding of CFRD pathophysiology and pathogenesis is incomplete, leading to variable standards of care.
  • Emerging evidence suggests CFRD may have developmental origins, with pancreatic pathology observed in utero in CF patients.

Purpose of the Study:

  • To review existing evidence on the role of the CF transmembrane conductance regulator (CFTR) in pancreatic development.
  • To explore the potential link between CFTR mutations, impaired pancreatic organogenesis, and the development of CFRD.
  • To highlight key constituents involved in pancreatic organogenesis, including endocrine and exocrine pancreas aspects and the islet microenvironment.

Main Methods:

  • Literature review of existing evidence.
  • Synthesis of data on CFTR function in pancreatic development.
  • Analysis of the impact of CFTR mutations on pancreatic organogenesis.

Main Results:

  • CFTR mutations may impair pancreatic organogenesis, contributing to CFRD development.
  • Evidence suggests a role for CFTR in the development of both endocrine and exocrine pancreatic functions.
  • The islet microenvironment is a critical factor in pancreatic development influenced by CFTR.

Conclusions:

  • CFTR plays a vital role in pancreatic development, and its dysfunction is implicated in CFRD pathogenesis.
  • Further research into the developmental origins of CFRD is crucial for advancing patient care.
  • Unraveling CFRD pathogenesis can lead to transformed future care strategies for individuals with cystic fibrosis.

Keywords:
Cystic fibrosisCystic fibrosis transmembrane conductance regulatorCystic fibrosis-related diabetesDevelopmentMicroenvironmentOrganogenesisPancreasReview

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