The impaired targeting to the plasma membrane of ATP8B1 caused by the most frequent ATP8B1 disease mutation in European patients, I661T, was recently shown to be rescued by compounds known to be efficient correctors of CFTR misfolding in cystic fibrosis, thus pointing to a possible therapeutic strategy (Van Der Woerd et al., 2016). Here, ATP8B1 is linked to cystic fibrosis.