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• W2062715512 abstract "α1-Antitrypsin α1-AT), an acute phase reactant glycoprotein consisting of a single polypeptide chain with four carbohydrate side chains, represents about 90% of the α1-fraction in human serum protein electrophoresis. It is a major inhibitor of proteolytic enzymes and shows a broad spectrum of antiproteolytic activity. Leukocyte collagenase and elastase probably represent its most important targets. Congenital deficiency of α1-AT associated with childhood liver cirrhosis and pulmonary emphysema in young adults was detected ten to fifteen years ago and stimulated detailed genetic, biochemical, and morphologic studies. A pair of polymorphic, completely penetrant, codominant autosomal alleles at the Pi locus determine the production of a number of α1-AT variants with different electrophoretic mobilities. Homozygous Pi M is the most common α1-AT phenotype, present in more than 90% of the general population. Severe deficiency of α1-AT is found in homozygous Pi Z, heterozygous Pi SZ, and in subjects carrying the Pi “null” allele. Biochemical investigations recently revealed amino acid substitution in the Pi Z and Pi S molecules, possibly leading to altered electrophoretic mobility and reduced excretion of variant molecules into circulation. Distinct PAS-positive, diastase-resistant globular inclusions in the cytoplasm of liver cells are a diagnostic feature of α1-AT deficiency Pi type Z. It has been shown immunohistochemically that the globules represent accumulations of al-AT, which is synthesized in the liver. Ultrastructurally the globules are located in the smooth and rough endoplasmic reticulum, suggesting that a defect in transportation may be responsible for both storage and low excretion into circulation. The pathogenesis of liver cirrhosis and pulmonary emphysema in severe α1-ATD is still a matter of debate. This appears to be mainly due to incomplete knowledge of the physiologic role of α1-AT and other proteases in human serum." @default.
• W2062715512 created "2016-06-24" @default.
• W2062715512 creator A5040826143 @default.
• W2062715512 date "1980-06-01" @default.
• W2062715512 modified "2023-09-28" @default.
• W2062715512 title "α1-Antitrypsin and its Deficiency States" @default.
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• W2062715512 doi "https://doi.org/10.1016/s0344-0338(80)80204-4" @default.
• W2062715512 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/7001411" @default.
• W2062715512 hasPublicationYear "1980" @default.
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