In vitro synthesis of M and Z forms of human α1‐antitrypsin

FEBS Letters

Janus, E.D.¹  Carrell, R.W.¹  Bathurst, I.C.¹  Errington, D.M.¹ 

[1] Molecular Pathology Laboratory, Pathology Department, Clinical School (University of Otago), Christchurch Hospital, Christchurch New Zealand

关键词: α1AT synthesis;    α1AT deficiency;    Human liver mRNA;   

DOI  :  10.1016/0014-5793(82)81247-7

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

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.

【 授权许可】

Unknown