CFTR High Expresser Cells in cystic fibrosis and intestinal diseases

Cystic Fibrosis Transmembrane Conductance Regulator (CFTR), the Cl−/HCO3− channel implicated in Cystic Fibrosis, is critical to the pathophysiology of many gastrointestinal diseases. Defects in CFTR lead to intestinal dysfunction, malabsorption, obstruction, infection, inflammation, and cancer that increases morbidity and reduces quality of life. This review will focus on CFTR in the intestine and the implications of the subpopulation of CFTR High Expresser Cells (CHEs) in Cystic Fibrosis (CF), intestinal physiology and pathophysiology of intestinal diseases.

• Publication year

  2023

• Venue

  Heliyon

• Publication date

  2023-03-01

• Fields of study

  Biology, Medicine

• Identifiers
  DOI 10.1016/j.heliyon.2023.e14568PMID 36967909PMCID 10031467
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• Receptor Guanylyl Cyclase C and Cyclic GMP in Health and Disease: Perspectives and Therapeutic Opportunities

  2022cited by this paper
• The Potential Role of Hypochlorhydria in the Development of Duodenal Dysbiosis: A Preliminary Report

  2022cited by this paper
• Obesity in Cystic fibrosis: prevalence, trends and associated factors data from the US cystic fibrosis foundation patient registry.

  2022cited by this paper
• Cancer statistics, 2022

  2022cited by this paper
• A novel peptide protects against diet-induced obesity by suppressing appetite and modulating the gut microbiota

  2022cited by this paper
• Association between Metabolic Syndrome and the Risk of Colorectal Cancer Diagnosed before 50 years According to Tumor Location.

  2022cited by this paper
• Loss of Serum Glucocorticoid-Inducible Kinase 1 SGK1 Worsens Malabsorption and Diarrhea in Microvillus Inclusion Disease (MVID)

  2022cited by this paper
• Global Cancer Statistics 2020: GLOBOCAN Estimates of Incidence and Mortality Worldwide for 36 Cancers in 185 Countries

  2021cited by this paper
• A Proximal-to-Distal Survey of Healthy Adult Human Small Intestine and Colon Epithelium by Single-Cell Transcriptomics

  2021cited by this paper
• Diagnosing constipation in patients with cystic fibrosis applying ESPGHAN criteria.

  2021cited by this paper
• Cystic Fibrosis and the Cells of the Airway Epithelium: What Are Ionocytes and What Do They Do?

  2021cited by this paper
• Cells of the human intestinal tract mapped across space and time

  2021cited by this paper
• Pendred Syndrome, or Not Pendred Syndrome? That Is the Question

  2021cited by this paper
• Human gastrointestinal epithelia of the esophagus, stomach, and duodenum resolved at single-cell resolution.

  2021cited by this paper
• Digenic inheritance of mutations in EPHA2 and SLC26A4 in Pendred syndrome

  2020cited by this paper
• Overweight, obesity and significant weight gain in adult patients with cystic fibrosis association with lung function and cardiometabolic risk factors.

  2020cited by this paper
• Editing Myosin VB Gene to Create Porcine Model of Microvillus Inclusion Disease, With Microvillus-lined Inclusions and Alterations in Sodium Transporters.

  2020cited by this paper
• Secretory Cells Dominate Airway CFTR Expression and Function in Human Airway Superficial Epithelia.

  2020cited by this paper
• Cell fate specification and differentiation in the adult mammalian intestine

  2020cited by this paper
• Inhibition of Na+/H+ exchanger isoform 3 improves gut fluidity and alkalinity in cystic fibrosis transmembrane conductance regulator‐deficient and F508del mutant mice

  2020cited by this paper
• Prevalence and factors associated with overweight and obesity in adults with cystic fibrosis: A single-center analysis.

  2019cited by this paper
• Association of Obesity With Risk of Early-Onset Colorectal Cancer Among Women

  2019cited by this paper
• Colonic epithelial cell diversity in health and inflammatory bowel disease

  2019cited by this paper
• Recent advances in understanding and managing malabsorption: focus on microvillus inclusion disease

  2019cited by this paper
• Distal renal tubular acidosis in mice that lack the forkhead transcription factor Foxi 1

  2018cited by this paper
• Differences in clinical outcomes of paediatric cystic fibrosis patients with and without meconium ileus.

  2018cited by this paper
• Spatial reconstruction of single enterocytes uncovers broad zonation along the intestinal villus axis

  2018cited by this paper
• Loss of MYO5B Leads to Reductions in Na+ Absorption With Maintenance of CFTR-Dependent Cl- Secretion in Enterocytes.

  2018cited by this paper
• Linaclotide improves gastrointestinal transit in cystic fibrosis mice by inhibiting sodium/hydrogen exchanger 3.

  2018cited by this paper
• A revised airway epithelial hierarchy includes CFTR-expressing ionocytes

  2018cited by this paper
• A single cell atlas of the tracheal epithelium reveals the CFTR-rich pulmonary ionocyte

  2018cited by this paper
• Guanylate cyclase 2C agonism corrects CFTR mutants.

  2017cited by this paper
• Cancer risk among lung transplant recipients with cystic fibrosis.

  2017cited by this paper
• Cystic Fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer.

  2017cited by this paper
• Meconium ileus in Cystic Fibrosis.

  2017cited by this paper
• Pendred syndrome.

  2017cited by this paper
• Molecular architecture underlying fluid absorption by the developing inner ear

  2017cited by this paper
• Linaclotide activates guanylate cyclase‐C/cGMP/protein kinase‐II‐dependent trafficking of CFTR in the intestine

  2017cited by this paper
• Identification of intestinal ion transport defects in microvillus inclusion disease.

  2016cited by this paper
• Guanylin and uroguanylin are produced by mouse intestinal epithelial cells of columnar and secretory lineage

  2016cited by this paper
• Ion channelopathies in functional GI disorders.

  2016cited by this paper
• Cellular localization of guanylin and uroguanylin mRNAs in human and rat duodenal and colonic mucosa

  2016cited by this paper
• Role of CFTR in epithelial physiology

  2016cited by this paper
• Calorie-induced ER stress suppresses uroguanylin satiety signaling in diet-induced obesity

  2016cited by this paper
• Overweight and obesity in patients with cystic fibrosis: A center‐based analysis

  2015cited by this paper
• Collecting duct intercalated cell function and regulation.

  2015cited by this paper
• Lineage-Specific Expression of Bestrophin-2 and Bestrophin-4 in Human Intestinal Epithelial Cells

  2013cited by this paper
• Regulated traffic of anion transporters in mammalian Brunner's glands: a role for water and fluid transport.

  2013cited by this paper
• Close Association of Carbonic Anhydrase (CA2a and CA15a), Na+/H+ Exchanger (Nhe3b), and Ammonia Transporter Rhcg1 in Zebrafish Ionocytes Responsible for Na+ Uptake

  2013cited by this paper
• GUCY2C: at the intersection of obesity and cancer.

  2013cited by this paper
• Characterization of CFTR High Expresser cells in the intestine.

  2013cited by this paper
• Obesity and colorectal cancer

  2013cited by this paper
• Myosin Ia is Required for CFTR Brush Border Membrane Trafficking and Ion Transport in the Mouse Small Intestine

  2012cited by this paper
• Cell-specific effects of luminal acid, bicarbonate, cAMP, and carbachol on transporter trafficking in the intestine.

  2012cited by this paper
• ATP secretion in the male reproductive tract: essential role of CFTR

  2012cited by this paper
• Genetic Evidence That Intestinal Notch Functions Vary Regionally and Operate through a Common Mechanism of Math1 Repression*

  2011cited by this paper
• Embryonic frog epidermis: a model for the study of cell-cell interactions in the development of mucociliary disease

  2011cited by this paper
• Physiological relevance of cell-specific distribution patterns of CFTR, NKCC1, NBCe1, and NHE3 along the crypt-villus axis in the intestine.

  2011cited by this paper
• Modulation of ion transporter expression in gill mitochondrion-rich cells of eels acclimated to low-Na(+) or-Cl(-) freshwater.

  2011cited by this paper
• A uroguanylin-GUCY2C endocrine axis regulates feeding in mice.

  2011cited by this paper
• Specification of ion transport cells in the Xenopus larval skin

  2011cited by this paper
• SAM pointed domain ETS factor (SPDEF) regulates terminal differentiation and maturation of intestinal goblet cells.

  2010cited by this paper
• Mechanism of development of ionocytes rich in vacuolar-type H(+)-ATPase in the skin of zebrafish larvae.

  2009cited by this paper
• The Forkhead Transcription Factor Foxi1 Is a Master Regulator of Vacuolar H+-ATPase Proton Pump Subunits in the Inner Ear, Kidney and Epididymis

  2009cited by this paper
• Inactivation of Notch signaling in the renal collecting duct causes nephrogenic diabetes insipidus in mice.

  2009cited by this paper
• Functional regulation of H+-ATPase-rich cells in zebrafish embryos acclimated to an acidic environment.

  2009cited by this paper
• Molecular physiology of bestrophins: multifunctional membrane proteins linked to best disease and other retinopathies.

  2008cited by this paper
• Gene expression and morphological localization of NHE3, NCC and NKCC1a in branchial mitochondria-rich cells of Mozambique tilapia (Oreochromis mossambicus) acclimated to a wide range of salinities.

  2008cited by this paper
• MYO5B mutations cause microvillus inclusion disease and disrupt epithelial cell polarity

  2008cited by this paper
• Intestinal Neurogenin 3 directs differentiation of a bipotential secretory progenitor to endocrine cell rather than goblet cell fate.

  2007cited by this paper
• Transcriptional control of SLC26A4 is involved in Pendred syndrome and nonsyndromic enlargement of vestibular aqueduct (DFNB4).

  2007cited by this paper
• Guanylyl cyclase C suppresses intestinal tumorigenesis by restricting proliferation and maintaining genomic integrity.

  2007cited by this paper
• Foxi3 transcription factors and Notch signaling control the formation of skin ionocytes from epidermal precursors of the zebrafish embryo.

  2007cited by this paper
• The forkhead transcription factor Foxi1 directly activates the AE4 promoter.

  2006cited by this paper
• Epididymal expression of the forkhead transcription factor Foxi1 is required for male fertility

  2006cited by this paper
• Overweight and obesity in deltaF508 homozygous cystic fibrosis.

  2005cited by this paper
• Gfi1 functions downstream of Math1 to control intestinal secretory cell subtype allocation and differentiation.

  2005cited by this paper
• Overweight and Obesity in ?F508 Homozygous Cystic Fibrosis

  2005cited by this paper
• Myosin-1a is critical for normal brush border structure and composition.

  2005cited by this paper
• CFTR in cystic fibrosis and cholera: from membrane transport to clinical practice.

  2005cited by this paper
• Guanylyl cyclase C is a marker of intestinal metaplasia, dysplasia, and adenocarcinoma of the gastrointestinal tract.

  2005cited by this paper
• Distal renal tubular acidosis in mice that lack the forkhead transcription factor Foxi1.

  2004cited by this paper
• A role for myosin-1A in the localization of a brush border disaccharidase

  2004cited by this paper
• Lack of pendrin expression leads to deafness and expansion of the endolymphatic compartment in inner ears of Foxi1 null mutant mice

  2003cited by this paper
• Pendred Syndrome

  2003cited by this paper
• Lieberkühn of the crypts of Lieberkühn.

  2003cited by this paper
• Cancer risk in nontransplanted and transplanted cystic fibrosis patients: a 10-year study.

  2003cited by this paper
• Site-specific effects of dietary salt intake on guanylin and uroguanylin mRNA expression in rat intestine.

  2002cited by this paper
• Requirement of Math1 for Secretory Cell Lineage Commitment in the Mouse Intestine

  2001cited by this paper
• Control of endodermal endocrine development by Hes-1

  2000cited by this paper
• Expression of GC-C, a receptor-guanylate cyclase, and its endogenous ligands uroguanylin and guanylin along the rostrocaudal axis of the intestine.

  2000cited by this paper
• Cellular localization of the cystic fibrosis transmembrane conductance regulator in mouse intestinal tract

  2000cited by this paper
• Guanylyl cyclases and signaling by cyclic GMP.

  2000cited by this paper
• Uroguanylin treatment suppresses polyp formation in the Apc(Min/+) mouse and induces apoptosis in human colon adenocarcinoma cells via cyclic GMP.

  2000cited by this paper
• Subcellular distribution of CFTR in rat intestine supports a physiologic role for CFTR regulation by vesicle traffic

  2000cited by this paper
• CFTR channel insertion to the apical surface in rat duodenal villus epithelial cells is upregulated by VIP in vivo.

  1999cited by this paper
• Regulation of intestinal uroguanylin/guanylin receptor-mediated responses by mucosal acidity.

  1997cited by this paper
• Uroguanylin is expressed by enterochromaffin cells in the rat gastrointestinal tract.

  1997cited by this paper
• Guanosine 3',5'-cyclic monophosphate-dependent protein kinase II mediates heat-stable enterotoxin-provoked chloride secretion in rat intestine.

  1997cited by this paper
• Prouroguanylin and proguanylin: purification from colon, structure, and modulation of bioactivity by proteases.

  1996cited by this paper

• Cystic fibrosis risk variants confer protection against inflammatory bowel disease

  2025cites this paper
• CFTR High Expresser BEST4+ cells are pH-sensing neuropod cells: new implications for intestinal physiology and Cystic Fibrosis disease

  2025cites this paper
• The role of Bifidobacterium animalis QC08 in antibiotic-associated diarrhea in mice

  2025cites this paper
• A Single-Cell Atlas of Transcriptome Changes in the Intestinal Epithelium at the Suckling-to-Weaning Transition in Male Rabbits

  2025cites this paper
• CFTR High Expresser BEST4+ cells are pH-sensing neuropod cells: new implications for intestinal physiology and Cystic Fibrosis disease.

  2025cites this paper
• BEST4+ cells in the intestinal epithelium

  2024influential citation
• NET-related gene signature for predicting AML prognosis

  2024cites this paper
• Impaired intestinal free fatty acid transport followed by chylomicron malformation, not pancreatic insufficiency, cause metabolic defects in cystic fibrosis

  2024cites this paper
• Cystic Fibrosis and Cancer: Unraveling the Complex Role of CFTR Gene in Cancer Susceptibility

  2023cites this paper