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(Chest. 1996;110:243S-247S.)
© 1996 American College of Chest Physicians

agr1-Antitrypsin Deficiency

A Conformational Disease

Robin W. Carrell MD, PhD1; David A. Lomas MD, PhD2; Sanjiv Sidhar PhD3; and Richard Foreman PhD3

1 From the Department of Haematology, University of Cambridge, MRC Centre, Cambridge, England
2 From the Department of Medicine, University of Cambridge, MRC Centre, Cambridge, England
3 From the Department of Physiology and Pharmacology, University of Southampton, Southampton, England

The serpin family of protease inhibitors, to which agr1-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 with the common Z variant, as well as other variants of agr1-antitrypsin. The understanding of the molecular mechanisms provides a satisfying explanation for the clinical findings associated with these deficiency variants.

Key Words: antitrypsin deficiency • protease inhibitors • serpins






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Copyright © 1996 by the American College of Chest Physicians.