6MWT 6-min walk test AAT 1-antitrypsin AATD 1-antitrypsin deficiency ALT alanine aminotransferase AST aspartate aminotransferase COPD chronic obstructive pulmonary disease CRP C-reactive protein ELF epithelial lining fluid ER endoplasmic reticulum ERAD endoplasmic reticulum-associated degradation FEV1 forced expiratory volume in 1 s GGT -glutamyltransferase HCC hepatocellular carcinoma NE neutrophil elastase NF-B nuclear factor-B TACE tumour necrosis factor--converting enzyme TNF tumour necrosis factor UPR unfolded protein response Common genotypes Pi*M wild-type AAT allele Pi*MM normal AAT genotype Pi*SZ genotype with compound heterozygous AAT mutation Pi*Z/Pi*S mutated AAT alleles Pi*ZZ classic, severe AATD genotype List of abbreviations