Structure and variation of human α 1 -antitrypsin

The sequence of alpha 1-antitrypsin is in keeping with its role as a tissue scavenger of leukocyte elastase. Two abnormal variants commonly present in Europeans cause a deficiency that predisposes them to a progressive loss of lung elasticity. The nature of the reactive centre helps explain why ciga...

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Bibliographic Details
Published in:	Nature (London) Vol. 298; no. 5872; pp. 329 - 334
Main Authors:	Carrell, Robin W, Jeppsson, Jan-Olof, Laurell, Carl-Bertil, Brennan, Stephen O, Owen, Maurice C, Vaughan, Lloyd, Boswell, D. Ross
Format:	Journal Article
Language:	English
Published:	England 22-07-1982
Subjects:	alpha 1-Antitrypsin - physiology alpha 1-Antitrypsin Deficiency Amino Acid Sequence Chemical Phenomena Chemistry Genetic Variation Humans Liver - pathology Liver Diseases - etiology Liver Diseases - pathology Lung Diseases - etiology Pancreatic Elastase - metabolism Phenotype Pulmonary Emphysema - etiology Tobacco Use Disorder
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Summary:	The sequence of alpha 1-antitrypsin is in keeping with its role as a tissue scavenger of leukocyte elastase. Two abnormal variants commonly present in Europeans cause a deficiency that predisposes them to a progressive loss of lung elasticity. The nature of the reactive centre helps explain why cigarette smoking greatly accelerates the onset and severity of this degenerative process to give the disease emphysema.
ISSN:	0028-0836 1476-4687
DOI:	10.1038/298329a0