CFTR and alpha 1-antitrypsin deficiency: As a large fraction of these mutant proteins (i.e., HLA-27, CFTR and AATD in AS, CF and AATD, respectively) typically misfold within the ER lumen, persistent activation of the UPR is thought to contribute to the disease inflammatory phenotype [140,141,142,143,144,145,146,147,148].