Article

Excessive ENaC-mediated sodium influx drives NLRP3 inflammasome-dependent autoinflammation in cystic fibrosis

2018-10-31

Abstract excerpt

Cystic Fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene and results in defective CFTR-mediated chloride transport, dysregulation of epithelial sodium channels (ENaC) and exaggerated innate immune responses. We tested the hypothesis that upregulation of ENaC drives autoinflammation in this complex monogenic disease. We show that monocytes from patients with...

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Literature Corpus work: 95e75eb3-1ed3-5112-a05b-6cbfacd063f0
DOI: 10.1101/458208

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