Article
Function of monocytes and monocyte-derived macrophages in α1-antitrypsin deficiency.
The European respiratory journal - 1 Feb 2015
Van't Wout Emily F A, van Schadewijk Annemarie, Lomas David A, Stolk Jan, Marciniak Stefan J, Hiemstra Pieter S
Abstract excerpt
α1-antitrypsin deficiency is the most widely recognised genetic disorder causing chronic obstructive pulmonary disease (COPD). Mutant Z α1-antitrypsin expression has previously been linked to intracellular accumulation and polymerisation of this proteinase inhibitor. Subsequently, this has been described to underlie an exaggerated endoplasmic reticulum stress response and enhanced nuclear factor-κB signalling....
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