CFTR dysfunction in the intestinal epithelium is sufficient to promote pathogenic expansion of E. coli and enhanced barrier permeability in cystic fibrosis
It is demonstrated that the intestinal epithelium plays a dominant role in fecal microbiome alterations in CF and that the resultant dysbiosis contributes to CF pathogenesis.
It is proposed that CF may provide a naturally occurring human context in which chronic epithelial stress sustains EMP‐like pressure, a concept that requires direct validation in human CF intestinal tissue.
B. Umashankar, Ines Pankonien, Margarida D. Amaral et al.· Cell Proliferation· 0 citations
BACKGROUND
Highly effective CFTR modulator therapy with elexacaftor-tezacaftor-ivacaftor (ETI) has revolutionized clinical outcomes in cystic fibrosis (CF), yet its effects on gut and lung microbiota, especially at the functional level, are poorly understood.
METHODS
In a 12-month prospective study, we enrolled 35 cl...
N. Bastón-Paz, C. García-Durán, Doĝukan Bayraktar et al.· Archivos de Bronconeumologia· 0 citations
ABSTRACT Fragile X syndrome (FXS) is the leading monogenic cause of autism spectrum disorder (ASD), caused by mutation in the Fmr1 gene. In addition to cognitive and behavioral challenges, FXS patients often experience altered gut microbiome-induced gastrointestinal (GI) problems. Evidence suggests that an altered gut...
Sabiha Alam, C. Shaughnessy, E. Lucas et al.· Msphere· 0 citations
Increasing evidence suggests that acquired dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel occurs as a result of cigarette smoke exposure in chronic obstructive pulmonary disease (COPD). CFTR-targeted therapies were developed to treat genetic CFTR defects in cystic fibrosis,...
Nikhil T. Awatade, K. Budden, P. Pathinayake et al.· International Journal of Mol...· 0 citations
Simple Summary Feline diarrhea is a common gastrointestinal disease in veterinary clinics. It is hypothesized that dysbiosis of Gram-negative bacteria serves as a critical predisposing factor for this disease; however, in vivo evidence confirming that such microbiota triggers intestinal inflammation and disrupts intest...
Jing Wang, Xin-Hui Zhang, Xin-Ran Song et al.· Veterinary Sciences· 0 citations
Cystic fibrosis (CF) is caused by pathogenic variants of the Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene. CF in the airways is associated with dehydrated and hyperconcentrated mucus, impaired mucociliary transport, and inflammation. Although CF is a genetic disorder, CFTR sequence variations alone m...
A. De Ganzó, Brian Chan, M. Rubinstein et al.· Journal of Cystic Fibrosis· 0 citations
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