Article
Alpha 1-antitrypsin deficiency. A conformational disease.
Chest - 1 Dec 1996
Carrell R W, Lomas D A, Sidhar S, Foreman R
Abstract excerpt
The serpin family of protease inhibitors, to which alpha 1-antitrypsin belongs, has the unique feature of a mobile reactive center. Mutations within the critical regions of the molecule that control this mobility can allow premature changes in conformation with consequent abnormalities in folding and accompanying polymer formation. These abnormalities explain the plasma deficiency and liver inclusions associated...
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