CFTR represses a PDX1 axis to govern pancreatic ductal cell fate

Summary: Inflammation, acinar atrophy, and ductal hyperplasia drive pancreatic remodeling in newborn cystic fibrosis (CF) ferrets lacking a functional cystic fibrosis conductance regulator (CFTR) channel. These changes are associated with a transient phase of glucose intolerance that involves islet...

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Main Authors: Pavana G. Rotti, Yaling Yi, Grace Gasser, Feng Yuan, Xingshen Sun, Idil Apak-Evans, Peipei Wu, Guangming Liu, Soon Choi, Rosie Reeves, Attilina E. Scioneaux, Yulong Zhang, Michael Winter, Bo Liang, Nathan Cunicelli, Aliye Uc, Andrew W. Norris, Lori Sussel, Kristen L. Wells, John F. Engelhardt
Format: Article
Language:English
Published: Elsevier 2024-12-01
Series:iScience
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Online Access:http://www.sciencedirect.com/science/article/pii/S258900422402618X
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author Pavana G. Rotti
Yaling Yi
Grace Gasser
Feng Yuan
Xingshen Sun
Idil Apak-Evans
Peipei Wu
Guangming Liu
Soon Choi
Rosie Reeves
Attilina E. Scioneaux
Yulong Zhang
Michael Winter
Bo Liang
Nathan Cunicelli
Aliye Uc
Andrew W. Norris
Lori Sussel
Kristen L. Wells
John F. Engelhardt
author_facet Pavana G. Rotti
Yaling Yi
Grace Gasser
Feng Yuan
Xingshen Sun
Idil Apak-Evans
Peipei Wu
Guangming Liu
Soon Choi
Rosie Reeves
Attilina E. Scioneaux
Yulong Zhang
Michael Winter
Bo Liang
Nathan Cunicelli
Aliye Uc
Andrew W. Norris
Lori Sussel
Kristen L. Wells
John F. Engelhardt
author_sort Pavana G. Rotti
collection DOAJ
description Summary: Inflammation, acinar atrophy, and ductal hyperplasia drive pancreatic remodeling in newborn cystic fibrosis (CF) ferrets lacking a functional cystic fibrosis conductance regulator (CFTR) channel. These changes are associated with a transient phase of glucose intolerance that involves islet destruction and subsequent regeneration near hyperplastic ducts. The phenotypic changes in CF ductal epithelium and their impact on islet function are unknown. Using bulk RNA sequencing (RNA-seq), single-cell RNA sequencing (scRNA-seq), and assay for transposase-accessible chromatin using sequencing (ATAC-seq) on CF ferret models, we demonstrate that ductal CFTR protein constrains PDX1 expression by maintaining PTEN and GSK3β activation. In the absence of CFTR protein, centroacinar cells adopted a bipotent progenitor-like state associated with enhanced WNT/β-Catenin, transforming growth factor β (TGF-β), and AKT signaling. We show that the level of CFTR protein, not its channel function, regulates PDX1 expression. Thus, this study has discovered a cell-autonomous CFTR-dependent mechanism by which CFTR mutations that produced little to no protein could impact pancreatic exocrine/endocrine remodeling in people with CF.
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spelling doaj-art-adf31ce97f1349c6913af02b6606f6922024-12-22T05:29:13ZengElsevieriScience2589-00422024-12-012712111393CFTR represses a PDX1 axis to govern pancreatic ductal cell fatePavana G. Rotti0Yaling Yi1Grace Gasser2Feng Yuan3Xingshen Sun4Idil Apak-Evans5Peipei Wu6Guangming Liu7Soon Choi8Rosie Reeves9Attilina E. Scioneaux10Yulong Zhang11Michael Winter12Bo Liang13Nathan Cunicelli14Aliye Uc15Andrew W. Norris16Lori Sussel17Kristen L. Wells18John F. Engelhardt19Whitehead Institute, MIT, Cambridge, MA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USADepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USAStead Family Department of Pediatrics, Carver College of Medicine, Iowa City, IA, USACenter for Gene Therapy, Carver College of Medicine, University of Iowa, Iowa City, IA, USABarbara Davis Center for Childhood Diabetes, University of Colorado Anschutz, Medical Campus, Aurora, CO, USABarbara Davis Center for Childhood Diabetes, University of Colorado Anschutz, Medical Campus, Aurora, CO, USA; Corresponding authorDepartment of Anatomy and Cell Biology, Carver College of Medicine, University of Iowa, Iowa City, IA, USA; Corresponding authorSummary: Inflammation, acinar atrophy, and ductal hyperplasia drive pancreatic remodeling in newborn cystic fibrosis (CF) ferrets lacking a functional cystic fibrosis conductance regulator (CFTR) channel. These changes are associated with a transient phase of glucose intolerance that involves islet destruction and subsequent regeneration near hyperplastic ducts. The phenotypic changes in CF ductal epithelium and their impact on islet function are unknown. Using bulk RNA sequencing (RNA-seq), single-cell RNA sequencing (scRNA-seq), and assay for transposase-accessible chromatin using sequencing (ATAC-seq) on CF ferret models, we demonstrate that ductal CFTR protein constrains PDX1 expression by maintaining PTEN and GSK3β activation. In the absence of CFTR protein, centroacinar cells adopted a bipotent progenitor-like state associated with enhanced WNT/β-Catenin, transforming growth factor β (TGF-β), and AKT signaling. We show that the level of CFTR protein, not its channel function, regulates PDX1 expression. Thus, this study has discovered a cell-autonomous CFTR-dependent mechanism by which CFTR mutations that produced little to no protein could impact pancreatic exocrine/endocrine remodeling in people with CF.http://www.sciencedirect.com/science/article/pii/S258900422402618XPhysiologyMolecular biologyCell biologyTranscriptomics
spellingShingle Pavana G. Rotti
Yaling Yi
Grace Gasser
Feng Yuan
Xingshen Sun
Idil Apak-Evans
Peipei Wu
Guangming Liu
Soon Choi
Rosie Reeves
Attilina E. Scioneaux
Yulong Zhang
Michael Winter
Bo Liang
Nathan Cunicelli
Aliye Uc
Andrew W. Norris
Lori Sussel
Kristen L. Wells
John F. Engelhardt
CFTR represses a PDX1 axis to govern pancreatic ductal cell fate
iScience
Physiology
Molecular biology
Cell biology
Transcriptomics
title CFTR represses a PDX1 axis to govern pancreatic ductal cell fate
title_full CFTR represses a PDX1 axis to govern pancreatic ductal cell fate
title_fullStr CFTR represses a PDX1 axis to govern pancreatic ductal cell fate
title_full_unstemmed CFTR represses a PDX1 axis to govern pancreatic ductal cell fate
title_short CFTR represses a PDX1 axis to govern pancreatic ductal cell fate
title_sort cftr represses a pdx1 axis to govern pancreatic ductal cell fate
topic Physiology
Molecular biology
Cell biology
Transcriptomics
url http://www.sciencedirect.com/science/article/pii/S258900422402618X
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