PubMed · 10716194
Cleaved antitrypsin polymers at atomic resolution.
Abstract
Alpha1-antitrypsin deficiency, which can lead to both emphysema and liver disease, is a result of the accumulation of alpha1-antitrypsin polymers within the hepatocyte. A wealth of biochemical and biophysical data suggests that alpha1-antitrypsin polymers form via insertion of residues from the reactive center loop of one molecule into the beta-sheet of another. However, this long-standing hypothesis has not been confirmed by direct structural evidence. Here, we describe the first crystallographic evidence of a beta-strand linked polymer form of alpha1-antitrypsin: the crystal structure of a cleaved alpha1-antitrypsin polymer.
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M A Dunstone, W Dai, J C Whisstock, J Rossjohn, R N Pike, S C Feil, B F Le Bonniec, M W Parker, S P Bottomley. 2000. Cleaved antitrypsin polymers at atomic resolution.. https://doi.org/10.1110/ps.9.2.417
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