Article
Z α1-antitrypsin confers a proinflammatory phenotype that contributes to chronic obstructive pulmonary disease.
American journal of respiratory and critical care medicine - 15 Apr 2014
Alam Samuel, Li Zhenjun, Atkinson Carl, Jonigk Danny, Janciauskiene Sabina, Mahadeva Ravi
Abstract excerpt
RATIONALE: Severe α1-antitrypsin deficiency caused by the Z variant (Glu342Lys; ZZ-AT) is a well-known genetic cause for emphysema. Although severe lack of antiproteinase protection is the critical etiologic factor for ZZ-AT-associated chronic obstructive pulmonary disease (COPD), some reports have suggested enhanced lung inflammation as a factor in ZZ-AT homozygotes. OBJECTIVES: To provide molecular...
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