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Alpha-1 antitrypsin deficiency alleles and severe cystic fibrosis lung disease

BACKGROUND—Alpha-1 antitrypsin (α(1)-AT) is the most abundant proteinase inhibitor within the lung. We have recently reported the surprising observation that cystic fibrosis patients with mild to moderate deficiency of α(1)-antitrypsin have significantly better pulmonary function than non-deficient...

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書目詳細資料
Main Authors: Mahadeva, R, Stewart, S, Bilton, D, Lomas, D
格式: Artigo
語言:Inglês
出版: BMJ Group 1998
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在線閱讀:https://ncbi.nlm.nih.gov/pmc/articles/PMC1745129/
https://ncbi.nlm.nih.gov/pubmed/10195072
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