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- W2000664686 abstract "An inherited deficiency of alpha1-antitrypsin (A1AT) in human serum predisposes to the development of pulmonary emphysema. A1AT is synthesized primarily by the liver, but no specific liver abnormality has been described in deficient subjects with lung disease. Fourteen of 17 livers from emphysematous patients with a Piz variant of A1AT (nine homozygotes and five heterozygotes) showed discrete, rounded, membrane enclosed cytoplasmic globules 1 to 20 microns in diameter and strongly PAS positive after diastase digestion. Three livers from patients with a Pis variant of A1AT did not have the globules. A battery of histochemical tests indicated a nonlipid, glycoprotein, globular composition. Electron microscopic study showed the globules to be single membrane bound, roughly oval, and occasionally irregular, containing a homogeneous, moderately electron dense substance. Application of fluorescein conjugated antisera to human A1AT to the liver tissues in five patients revealed positively staining structures corresponding to the globules. Use of fluorescein conjugated antisera to human albumin, fibrinogen, and gamma globulin revealed no globular fluorescence. These observations document the presence of an intracellular hepatic accumulation of A1AT in patients with the serum deficiency and emphysema and complement the findings of Sharp, who has described similar globules in inherited infantile cirrhosis and A1AT deficiency. No similar structures were observed in the livers of the emphysematous patients with a normal or elevated A1AT level. The cause of this accumulation may be an anomalous organelle, an allelic variant that is unable to escape, or a third unidentified factor." @default.
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- W2000664686 date "1972-09-01" @default.
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- W2000664686 title "Alpha1-antitrypsin (A1AT) accumulation in livers of emphysematous patients with A1AT deficiency" @default.
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- W2000664686 doi "https://doi.org/10.1016/s0046-8177(72)80037-6" @default.
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