In vitro synthesis of M and Z forms of human α1‐antitrypsin
- 1 November 1982
- journal article
- Published by Wiley in FEBS Letters
- Vol. 148 (1) , 83-86
- https://doi.org/10.1016/0014-5793(82)81247-7
Abstract
MRNA was prepared from autopsy liver samples from a homozygote for α1‐antitrypsin deficiency (PiZZ) and from a normal (PiMM) subject. Both preparation gave equivalent synthesis of α1‐antitrypsin in a wheat germ cell‐free system. This suggests that the deficiency of plasma α1‐antitrypsin associated with the Z variant is due to a failure of processing and secretion of the protein rather than of its synthesis. It is likely that it is the resultant intracellular accumulation of the Z protein rather than a deficiency of protease inhibitor that is the primary cause of the liver pathology associated with this variant.Keywords
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