In vitro synthesis of M and Z forms of human α 1 ‐antitrypsin
1982; Wiley; Volume: 148; Issue: 1 Linguagem: Inglês
10.1016/0014-5793(82)81247-7
ISSN1873-3468
AutoresD.M. Errington, Ian Bathurst, E.D. Janus, Robin W. Carrell,
Tópico(s)Enzyme Production and Characterization
ResumomRNA was prepared from autopsy liver samples from a homozygote for α 1 ‐antitrypsin deficiency ( PiZZ ) and from a normal ( PiMM ) subject. Both preparation gave equivalent synthesis of α 1 ‐antitrypsin in a wheat germ cell‐free system. This suggests that the deficiency of plasma α 1 ‐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.
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