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Immunologic assay of alpha1-antitrypsin in deficient subjects and their families

✍ Scribed by F. Kueppers

Publisher
Springer
Year
1967
Tongue
English
Weight
406 KB
Volume
5
Category
Article
ISSN
0340-6717

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## Abstract Alpha 1 antitrypsin phenotypes and serum levels are presented for a family in which two brothers have Weber Christian disease and Ξ±1 antitrypsin (PI) Z phenotypes. Clinical histories are described for these two men. A younger brother has the PI Z phenotype but does not have the disease,

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Alpha-1 antitrypsin (AAT; HUGO symbol, SERPINA1) is one of the major serine protease inhibitors (serpins) in human plasma. Deficiency of AAT is a recognised risk factor for chronic obstructive pulmonary disease (COPD), attributed to uninhibited neutrophil elastase released into the lung tissue durin