Human Z alpha 1-antitrypsin accumulates intracellularly and stimulates lysosomal activity when synthesised in the Xenopus oocyte.
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.
D000818:Animals; D005260:Female; D006358:Hot Temperature; D006801:Humans; D008099:Liver / Q000032:analysis; D008247:Lysosomes / Q000201:enzymology*; D008845:Microinjections; D009865:Oocytes / Q000187:drug effects / Q000378:metabolism*; D012333:RNA, Messenger / Q000494:pharmacology; D014982:Xenopus laevis; D000515:alpha 1-Antitrypsin / Q000096:biosynthesis / Q000235:genetics*
D012333:RNA, Messenger; D000515:alpha 1-Antitrypsin