CFTR and cystic fibrosis: CFTR dysfunction has been reported to contribute to abnormal HE4 expression via the activation of the pro-inflammatory NF-κB pathway in CF, and the effect of LUM/IVA treatment on HE4 expression has been evaluated in CFBE 41o- cells expressing p.Phe508del-CFTR in vitro, thereby attesting to the downregulation of the aforementioned pro-inflammatory pathway [18].