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α‎1-Antitrypsin deficiency and the serpinopathies 

α‎1-Antitrypsin deficiency and the serpinopathies
α‎1-Antitrypsin deficiency and the serpinopathies

David A. Lomas


July 30, 2015: This chapter has been re-evaluated and remains up-to-date. No changes have been necessary.


New and emerging treatments—discussion of use of carbamazepine, genomically corrected fibroblasts, and strategies to ‘knock down’ the expression of mutant Z α‎1-antitrypsin within hepatocytes.

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date: 24 September 2021

α‎1-Antitrypsin is an acute phase glycoprotein synthesized by the liver that functions as an inhibitor of a range of proteolytic enzymes, most importantly neutrophil elastase. Severe plasma deficiency of α‎1-antitrypsin results from homozygocity for the Z allele, which causes the protein to undergo a conformational transition and form ordered polymers that are retained within hepatocytes as PAS-positive inclusions....

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