Furthermore, a burgeoning number of studies now ascertain the crucial role of mature CFTR in regulating important cellular homeostatic processes such as proteostasis and autophagy, with a common consensus that autophagy is potentially inherently defective in CF (Gomes-Alves et al., 2010; Luciani et al., 2010, 2011; Bodas et al., 2012; Valle and Vij, 2012; Villella et al., 2013a). Here, CFTR is linked to cystic fibrosis.