The serpinopathies are a group of inherited disorders that share as their molecular basis the
misfolding and polymerization of serpins, an important class of protease inhibitors.
Depending on the identity of the serpin, conditions arising from polymerization include
emphysema, thrombosis, and dementia. The structure of serpin polymers is thus of
considerable medical interest. Wild-type α 1-antitrypsin will form polymers upon incubation
at moderate temperatures and has been widely used as a model system for studying serpin …