Since the loss-of-function mutations of ADGRG2 are responsible for an X-linked iCBAVD phenotypically similar to a CF-CBAVD, it has been assumed that CFTR and ADGRG2 may be involved in a common pathophysiological mechanism (Patat et al. 2016). Kirchoff et al. showed that there is a high expression of ADGRG2 in the microvilli of non-ciliated cells of the efferent ductules where it co-locates with the F-actin-ezrin scaffold (Kirchhoff et al. 2008). This evidence concerns the gene ADGRG2 and cystic fibrosis.