Monocytes and Macrophages in Alpha-1 Antitrypsin Deficiency

Alpha-1 antitrypsin deficiency (AATD) is a genetic condition characterised by low circulating levels of alpha-1 antitrypsin (AAT), a serine proteinase inhibitor. The most common deficiency variants are the S and Z mutations, which cause the accumulation of misfolded AAT in hepatocytes resulting in e...

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Veröffentlicht in:	International journal of chronic obstructive pulmonary disease 2020, Vol.15, p.3183-3192
Hauptverfasser:	Belchamber, Kylie B R, Walker, Eloise M, Stockley, Robert A, Sapey, Elizabeth
Format:	Artikel
Sprache:	eng
Schlagworte:	Antigen presentation Cytokines Development and progression Infections Inflammation Liver Liver diseases Lung diseases Lungs Macrophages monocyte macrophage alpha-1 antitrypsin alpha-1 antitrypsin deficiency Mutation Neutrophils Physiology Polymers Proteases Proteins Recruitment Review
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Beschreibung
Zusammenfassung:	Alpha-1 antitrypsin deficiency (AATD) is a genetic condition characterised by low circulating levels of alpha-1 antitrypsin (AAT), a serine proteinase inhibitor. The most common deficiency variants are the S and Z mutations, which cause the accumulation of misfolded AAT in hepatocytes resulting in endoplasmic reticular stress and insufficient release of AAT into the circulation (
ISSN:	1178-2005 1176-9106 1178-2005
DOI:	10.2147/COPD.S276792