Molecular Mechanism of Z α1-Antitrypsin Deficiency
نویسندگان
چکیده
منابع مشابه
Molecular Mechanism of Z α1-Antitrypsin Deficiency*
The Z mutation (E342K) of α1-antitrypsin (α1-AT), carried by 4% of Northern Europeans, predisposes to early onset of emphysema due to decreased functional α1-AT in the lung and to liver cirrhosis due to accumulation of polymers in hepatocytes. However, it remains unclear why the Z mutation causes intracellular polymerization of nascent Z α1-AT and why 15% of the expressed Z α1-AT is secreted in...
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could have been generated during the chloroform-phe-no1 extraction because the internal standard was not added until after the enzymatic hydrolysis and the use of phenol for DNA extraction has been associated with artefactual generation of 8-hydroxy-2'-deoxyguano-sine. Furthermore, some readers might object to the use of ascorbic acid as an antioxidant because iron-ascorbate system s have often...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 2016
ISSN: 0021-9258
DOI: 10.1074/jbc.m116.727826