Abstract:The aim of this review article is to collate recent contributions of proteomic studies to cystic fibrosis transmembrane conductance regulator (CFTR) biology. We summarize advances from these studies and create an accessible resource for future CFTR proteomic efforts. We focus our attention on the CFTR interaction network at the cell surface, thus generating a CFTR ‘surfaceome’. We review the main findings about CFTR interactions and highlight several functional categories amongst these that could lead to the d… Show more
“…CFTR interacts with many membrane transporters to regulate epithelial function ( Iazzi et al, 2023 ). For example, CFTR modulates the activity of the Na + /H + exchanger (NHE) ( Ahn et al, 2001 ) and Cl − /HCO 3 − exchanger ( Lee et al, 1999 ; Ko et al, 2004 ) to adjust extra- and intracellular pH, implicating the function of these transporters in ASL pH regulation.…”
Section: Cftr-mediated Regulation Of Membrane Transporters and Intrac...mentioning
Dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel by genetic mutations causes the inherited disease cystic fibrosis (CF). CF lung disease that involves multiple disorders of epithelial function likely results from loss of CFTR function as an anion channel conducting chloride and bicarbonate ions and its function as a cellular regulator modulating the activity of membrane and cytosol proteins. In the absence of CFTR activity, abundant mucus accumulation, bacterial infection and inflammation characterize CF airways, in which inflammation-associated tissue remodeling and damage gradually destroys the lung. Deciphering the link between CFTR dysfunction and bacterial infection in CF airways may reveal the pathogenesis of CF lung disease and guide the development of new treatments. Research efforts towards this goal, including high salt, low volume, airway surface liquid acidosis and abnormal mucus hypotheses are critically reviewed.
“…CFTR interacts with many membrane transporters to regulate epithelial function ( Iazzi et al, 2023 ). For example, CFTR modulates the activity of the Na + /H + exchanger (NHE) ( Ahn et al, 2001 ) and Cl − /HCO 3 − exchanger ( Lee et al, 1999 ; Ko et al, 2004 ) to adjust extra- and intracellular pH, implicating the function of these transporters in ASL pH regulation.…”
Section: Cftr-mediated Regulation Of Membrane Transporters and Intrac...mentioning
Dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel by genetic mutations causes the inherited disease cystic fibrosis (CF). CF lung disease that involves multiple disorders of epithelial function likely results from loss of CFTR function as an anion channel conducting chloride and bicarbonate ions and its function as a cellular regulator modulating the activity of membrane and cytosol proteins. In the absence of CFTR activity, abundant mucus accumulation, bacterial infection and inflammation characterize CF airways, in which inflammation-associated tissue remodeling and damage gradually destroys the lung. Deciphering the link between CFTR dysfunction and bacterial infection in CF airways may reveal the pathogenesis of CF lung disease and guide the development of new treatments. Research efforts towards this goal, including high salt, low volume, airway surface liquid acidosis and abnormal mucus hypotheses are critically reviewed.
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