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1. Human Z alpha 1-antitrypsin accumulates intracellularly and stimulates lysosomal activity when synthesised in the Xenopus oocyte.

Author

Bathurst IC, Errington DM, Foreman RC, Judah JD, and Carrell RW

Subjects
Animals, Female, Hot Temperature, Humans, Liver analysis, Microinjections, Oocytes drug effects, RNA, Messenger pharmacology, Xenopus laevis, alpha 1-Antitrypsin biosynthesis, Lysosomes enzymology, Oocytes metabolism, alpha 1-Antitrypsin genetics
Abstract

Microinjection of human liver mRNA from a patient homozygous for alpha 1-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 (alpha 1-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.

Published
1985
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