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

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.

journal_name

FEBS Lett

journal_title

FEBS letters

authors

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

doi

10.1016/0014-5793(85)80798-5

subject

Has Abstract

pub_date

1985-04-22 00:00:00

pages

304-8

issue

2

eissn

0014-5793

issn

1873-3468

pii

0014-5793(85)80798-5

journal_volume

183

pub_type

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