Unknown,Transcriptomics,Genomics,Proteomics

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Transcription profiling of mouse transgenics (3 mo) inducibly expresssing human alpha1-antitrypsin in the liver


ABSTRACT: In the classical form of α1antitrypsin 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 α1antitrypsin 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 α1antitrypsin 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. Experiment Overall Design: Liver RNA from adult (3 mo old) male mice inducibly expressing human alpha1-antitrypsin wild type (M) or mutant (Z) form exclusively in the liver was subjected to genomic analysis. Groups: mutant AAT (Z), wild type AAT (M), expressing (4), non-expressing (1), wild type littermates (WT); 3 biological replicates/each group

ORGANISM(S): Mus musculus

SUBMITTER: Tunda Hidvegi 

PROVIDER: E-GEOD-8599 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress

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Publications

Regulator of G Signaling 16 is a marker for the distinct endoplasmic reticulum stress state associated with aggregated mutant alpha1-antitrypsin Z in the classical form of alpha1-antitrypsin deficiency.

Hidvegi Tunda T   Mirnics Karoly K   Hale Pamela P   Ewing Michael M   Beckett Caroline C   Perlmutter David H DH  

The Journal of biological chemistry 20070717 38


In the classical form of alpha(1)-antitrypsin deficiency, a mutant protein accumulates in a polymerized form in the endoplasmic reticulum (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 alpha(1)-antitrypsin 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 alpha(1)-antitryp  ...[more]

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