Human Z α1‐antitrypsin accumulates intracellularly and stimulates lysosomal activity when synthesised in the Xenopus oocyte

FEBS Letters

Foreman, R.C.²  Carrell, R.W.¹  Bathurst, I.C.¹  Errington, D.M.¹  Judah, J.D.² 

[1] Molecular Pathology Laboratory, Clinical School of Medicine (University of Otago), Christchurch Hospital, Christchurch, New Zealand;Department of Experimental Pathology. School of Medicine, University College London, University Street, London WC1E 6JJ, England

关键词: Lysosomal protein;    Oocyte injection;    Protein synthesis;    Protein secretion;    Z human α1-antitrypsin;   

DOI  :  10.1016/0014-5793(85)80798-5

学科分类：生物化学/生物物理

来源: John Wiley & Sons Ltd.

【 摘 要 】

Microinjection of human liver mRNA from a patient homozygous for α₁-antitrypsin deficiency (PiZZ) into Xenopus oocytes led to a 2–10-fold increase in lysosomal activity. Stimulation of lysosomal activity was not observed when mRNA from a normal human liver (α₁-antitrypsin PiMM), or water was injected into the oocyte. This lysosomal activity was oocyte derived and was not due to translation products of the human liver mRNA. Thus a protein that accumulates intracellularly in the secretory pathway is capable of stimulating lysosomal activity.

【 授权许可】

Unknown