Protein sorting and proteostasis mechanisms in CFTR-related exocrine pancreas dysfunction: A systematic narrative review.
Aime Patrick Niyomugabo, Leopold Ntakirutimana, Abdullateef Isiaka Alagbonsi
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In 30 seconds
This systematic narrative review examines the role of protein sorting mechanisms in CFTR-related exocrine pancreatic dysfunction. It synthesizes findings from 20 studies, highlighting that pathogenic CFTR variants disrupt protein folding and trafficking, leading to impaired bicarbonate secretion and acinar injury. The review emphasizes the need for clinical confirmation of these findings.
Key findings
- Pathogenic CFTR variants impair protein folding and endoplasmic reticulum exit.
- Reduced apical membrane expression of CFTR leads to altered zymogen handling.
- CFTR loss disrupts localization of associated transporters and secretory proteins.
- Therapeutic strategies targeting channel correction may improve pancreatic function.
Why it matters
Understanding the mechanisms of CFTR-related dysfunction in the exocrine pancreas is crucial for developing targeted therapies. Improved insights into protein sorting and trafficking could lead to better management strategies for patients with CFTR channelopathies.