Interestingly, A1AT contains a 188Y-I-F-F191 motif and preliminary data from our laboratory suggest ERp29 and A1AT may interact via this binding domain in co-precipitation experiments in Madin-Darby Canine Kidney (MDCK) and Cystic Fibrosis Bronchial Epithelial (CFBE41o-) cells (unpublished data). Here, SERPINA1 is linked to cystic fibrosis.