Journal article
α1-Antitrypsin Deficiency Alleles in Cystic Fibrosis Lung Disease
Abstract
Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) genotype does not explain the heterogeneity observed in CF pulmonary disease severity. Modifier genes are implicated for this heterogeneity. alpha1-antitrypsin (alpha1-AT) is one of the few antiproteases capable of inactivating neutrophil elastase. We investigated whether alpha1-AT alleles (Z, S deficiency alleles and the 3' G1237-->A mutation) were associated with increased …
Authors
Frangolias DD; Ruan J; Wilcox PJ; Davidson AGF; Wong LTK; Berthiaume Y; Hennessey R; Freitag A; Pedder L; Corey M
Journal
American Journal of Respiratory Cell and Molecular Biology, Vol. 29, No. 3, pp. 390–396
Publisher
American Thoracic Society
Publication Date
September 2003
DOI
10.1165/rcmb.2002-0271oc
ISSN
1044-1549