Hepatic Changes in Adult α1-Antitrypsin Deficiency
نویسندگان
چکیده
منابع مشابه
α1-Antitrypsin deficiency: count me in please!
Respiratory medicine is facing formidable challenges in the 21st century. Indeed, most respiratory medical research is becoming interconnected, translational and transnational, all embedded within so-called “planetary health” [1]. However, renewing estimates and trends from classical, descriptive epidemiology, including how many patients of a given condition are around, and how many are expecte...
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Most individuals carry two wild-type M alleles of the SERPINA1 gene which encodes a1-antitrypsin. 95% of severe deficiency of a1-antitrypsin is associated with the Z allele (Glu342Lys; denoted PiZZ in the homozygote), and with the retention and polymerisation of a1-antitrypsin within hepatocytes [1]. These polymers are contained within periodic acid–Schiff-positive, diastase-resistant inclusion...
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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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www.thelancet.com Published online May 28, 2015 http://dx.doi.org/10.1016/S0140-6736(15)60036-8 1 α1 antitrypsin is a serine antiprotease secreted by the liver. Its main function is inhibition of neutrophil elastase, a non-specifi c protease released by activated or dying neutrophils. In individuals with healthy concentrations of α1 antitrypsin, the fragile alveolar structures of the lung paren...
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deficiency David J.A. Jenkins, MD, PhD, FRCP, Michael Freeman, MD, FRCPC, Iqwal Mangat, MD, FRCPC, Koruba Srichaikul, MD, MSc, Viranda H. Jayalath, MSc, Dorothea Faulkner, RD, PhD, John L. Sievenpiper, MD, PhD, FRCPC, Cyril W.C. Kendall, PhD, Alexander Romaschin, PhD, Young-In Kim, MD, FRCPC, Paul Dorian, MD, FRCPC, FHRS From the Department of Nutritional Sciences, Faculty of Medicine, Universi...
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ژورنال
عنوان ژورنال: Gastroenterology
سال: 1973
ISSN: 0016-5085
DOI: 10.1016/s0016-5085(19)33110-5