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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Veröffentlicht in:Nature (London) 1982-07, Vol.298 (5872), p.329-334
Hauptverfasser: Carrell, Robin W, Jeppsson, Jan-Olof, Laurell, Carl-Bertil, Brennan, Stephen O, Owen, Maurice C, Vaughan, Lloyd, Boswell, D. Ross
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container_end_page 334
container_issue 5872
container_start_page 329
container_title Nature (London)
container_volume 298
creator Carrell, Robin W
Jeppsson, Jan-Olof
Laurell, Carl-Bertil
Brennan, Stephen O
Owen, Maurice C
Vaughan, Lloyd
Boswell, D. Ross
description 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.
doi_str_mv 10.1038/298329a0
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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
title Structure and variation of human α 1 -antitrypsin
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