Xenopus oocytes can synthesise but do not secrete the Z variant of human alpha 1-antitrypsin |
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Authors: | R C Foreman J D Judah A Colman |
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Affiliation: | 1. Department of Experimental Pathology, University College London, University Street, London WC1E 6JJ, England;2. Department of Biological Sciences, University of Warwick, Coventry CV4 7AL, England |
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Abstract: | Human liver mRNA was prepared from a patient homozygous for alpha 1-antitrypsin deficiency (PiZZ) and from a normal subject (PiMM). Both liver RNAs were microinjected into Xenopus oocytes and alpha 1-antitrypsin identified by immunoprecipitation. The normal M variant of alpha 1-antitrypsin is synthesised and secreted by Xenopus oocytes, the abnormal Z protein is not secreted and an intracellular form accumulates in the oocytes. In the presence of tunicamycin an unglycosylated form of M alpha 1-antitrypsin appears in the incubation medium but no corresponding unglycosylated version of the Z protein is secreted. |
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Keywords: | Human liver mRNA PiZ variant |
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