Alpha-1 antitrypsin deficiency (AATD) is most often caused by the Z mutant variant of AAT (ZAAT), a deficiency allele of the SERPINA1 gene [3], resulting in misfolding, aggregation, and accumulation of ZAAT aggregates followed by endoplasmic reticulum (ER) stress in the hepatocytes [4,5]. Here, SERPINA1 is linked to alpha 1-antitrypsin deficiency.