Point mutations cause members of the serine protease inhibitor (serpin) superfamily to
undergo a novel conformational transition, forming ordered polymers. These polymers …

Alpha-1 antitrypsin deficiency is an inherited disease affecting the lung and liver. The typical
pulmonary manifestation is chronic obstructive pulmonary disease and emphysema. Severe …

Alpha1‐antitrypsin is the most abundant circulating protease inhibitor. The severe Z
deficiency allele (Glu342Lys) causes the protein to undergo a conformational transition and …

The serpinopathies result from the ordered polymerization of mutants of members of the
serine proteinase inhibitor (serpin) superfamily. These polymers are retained within the cell …

The Z mutant of α1-antitrypsin (Glu342Lys) causes a domain swap and the formation of
intrahepatic polymers that aggregate as inclusions and predispose the homozygote to …

The S-and Z-deficiency alleles of α1-antitrypsin are found in more than 20% of some white
populations. This high gene frequency suggests that these mutations confer a selective …

Although protein folding is a simple outcome of the underlying thermodynamics, arriving at a
quantitative and predictive understanding of how proteins fold nevertheless poses huge …

Metastasis is a major obstacle that must be overcome for the successful treatment of lung
cancer. Proteins secreted by cancer cells may facilitate the progression of metastasis …

Protein misfolding is a process in which proteins are unable to attain or maintain their
biologically active conformation. Factors contributing to protein misfolding include missense …

α1-Antitrypsin is the prototypical member of the serine proteinase inhibitor or serpin
superfamily of proteins. The family includes α1-antichymotrypsin, C1 inhibitor, antithrombin …