CFTR High Expresser Cells in cystic fibrosis and intestinal diseases

Diego Carlos Dos Reis; Parinaz Dastoor; Anderson Kenedy Santos; Kaelyn Sumigray; Nadia A Ameen

doi:10.1016/j.heliyon.2023.e14568

CFTR High Expresser Cells in cystic fibrosis and intestinal diseases

Heliyon. 2023 Mar 14;9(3):e14568. doi: 10.1016/j.heliyon.2023.e14568. eCollection 2023 Mar.

Authors

Diego Carlos Dos Reis¹, Parinaz Dastoor¹, Anderson Kenedy Santos^{1

2}, Kaelyn Sumigray^{2

3

4}, Nadia A Ameen^{1

5}

Affiliations

¹ Department of Pediatrics/Gastroenterology and Hepatology, Yale School of Medicine, CT, 06510, USA.
² Department of Genetics, Yale School of Medicine, New Haven, CT, 06510, USA.
³ Yale Stem Cell Center, Yale School of Medicine, New Haven, CT, 06510, USA.
⁴ Yale Cancer Center, Yale School of Medicine, New Haven, CT, 06510, USA.
⁵ Department of Cellular and Molecular Physiology, Yale School of Medicine, New Haven, CT06510, USA.

Abstract

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.

Keywords: CFTR; CFTR high expresser cells; Cystic fibrosis; Intestinal disease; Ionocytes; Mitochondria rich cells.

Publication types

Review

Abstract

Publication types

Grants and funding