Article
alpha1-Antitrypsin deficiency, chronic obstructive pulmonary disease and the serpinopathies.
Clinical science (London, England : 1979) - 14 May 2009
Ekeowa Ugo I, Gooptu Bibek, Belorgey Didier, Hägglöf Peter, Karlsson-Li Susanna, Miranda Elena, Pérez Juan, MacLeod Ian, Kroger Heike, Marciniak Stefan J, Crowther Damian C, Lomas David A
Abstract excerpt
alpha1-Antitrypsin is the prototypical member of the serine proteinase inhibitor or serpin superfamily of proteins. The family includes alpha1-antichymotrypsin, C1 inhibitor, antithrombin and neuroserpin, which are all linked by a common molecular structure and the same suicidal mechanism for inhibiting their target enzymes. Point mutations result in an aberrant conformational transition and the formation of...
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