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Accession IconGSE8599

Expression data from transgenic mice (3 mo) inducibly expressing human alpha1-antitrypsin in the liver

Organism Icon Mus musculus
Sample Icon 15 Downloadable Samples
Technology Badge Icon Affymetrix Mouse Expression 430A Array (moe430a)

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Description
In the classical form of 1antitrypsin deficiency a mutant protein accumulates in a polymerized form in the ER of liver cells causing liver damage and carcinogenesis by a gain-of-toxic function mechanism. Recent studies have indicated that the accumulation of mutant 1antitrypsin Z in the ER specifically activates the autophagic response but not the unfolded protein response and that autophagy plays a critical role in disposal of insoluble 1antitrypsin Z. In this study, we used genomic analysis of the liver in a novel transgenic mouse model with inducible expression to screen for changes in gene expression that would potentially define how the liver responds to accumulation of this mutant protein.
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