Carlos Dos Reis Diego, Dastoor Parinaz, Santos Anderson Kenedy, Sumigray Kaelyn, Ameen Nadia A
Department of Pediatrics/Gastroenterology and Hepatology, Yale School of Medicine, CT, 06510, USA.
Department of Genetics, Yale School of Medicine, New Haven, CT, 06510, USA.
Heliyon. 2023 Mar 14;9(3):e14568. doi: 10.1016/j.heliyon.2023.e14568. eCollection 2023 Mar.
Cystic Fibrosis Transmembrane Conductance Regulator (CFTR), the Cl/HCO 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.
囊性纤维化跨膜传导调节因子(CFTR)是一种与囊性纤维化相关的Cl⁻/HCO₃⁻通道,对许多胃肠道疾病的病理生理学至关重要。CFTR缺陷会导致肠道功能障碍、吸收不良、梗阻、感染、炎症和癌症,从而增加发病率并降低生活质量。本综述将聚焦于肠道中的CFTR以及CFTR高表达细胞亚群(CHEs)在囊性纤维化(CF)、肠道生理学和肠道疾病病理生理学中的意义。