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Gene Expression Levels in PiZ mice Compared to Wild-type (Wt)C57Bl/6

ABSTRACT: Individuals expressing alpha-1-antitrypsin mutant Z protein accumulate misfolded, mutant protein in the liver and are at risk for liver diseases including cirrhosis and hepatocellular carcinoma. Transgenic PiZ mice, a model for this liver disease, display similar pathologies to humans, including inflammation, increases in proliferation, autophagy and apoptosis, accumulation of globules and develop fibrosis and hepatocellular carcinoma with age. Microarrays were used to compare the gene expressions of PiZ mice to wild-type mice in order to identify the pathways that are altered in this disorder.

ORGANISM(S): Mus musculus

PROVIDER: GSE36478 | GEO | 2012/11/08

SECONDARY ACCESSION(S): PRJNA153511

REPOSITORIES: GEO

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Gene Expression Levels in PiZ mice Compared to Wild-type (Wt)C57Bl/6

Project description:Individuals expressing alpha-1-antitrypsin mutant Z protein accumulate misfolded, mutant protein in the liver and are at risk for liver diseases including cirrhosis and hepatocellular carcinoma. Transgenic PiZ mice, a model for this liver disease, display similar pathologies to humans, including inflammation, increases in proliferation, autophagy and apoptosis, accumulation of globules and develop fibrosis and hepatocellular carcinoma with age. Microarrays were used to compare the gene expressions of PiZ mice to wild-type mice in order to identify the pathways that are altered in this disorder. Pooled samples from 4 mice, 3-4 months old were used for each of 4 categories. PiZ males, PiZ females, C57Bl/6 males, C57Bl/6 females

2012-11-08 | E-GEOD-36478 | biostudies-arrayexpress

Gene expression profile of wild type, PiZ and PiZ/Chop-/- mouse livers at two different ages

Project description:To investigate the role of CHOP in the pathogenesis of liver disease due to Z alpha-1 antitrypsin in juvenile and older mice, we performed Quant-seq experiments at 6 and 36 weeks of age in wild type, PiZ and PiZ/Chop-/- mouse livers

2020-08-13 | GSE141593 | GEO

Differential gene expression in ATZ-globule-free and ATZ-globule-containing hepatocytes in PIZ mouse liver

Project description:Alpha-1-antitrypsin deficiency is a disease of the liver and lung where accumulation of misfolded ATZ protein forms large protein aggregates or globules that result in significant cellular stess. From Birth to 2 months of age, virtually every hepatocyte has numerous globules of the mutant ATZ protien. At 2 months of age small colonies of globule free hepatocytes arise and over time the liver becomes largely globule free. this analysis aims to identify differential gene expression profiles in the globule free vs the globule containing cells to idenitfy key factors and pathways regulating clearance of the mutant ATZ protien. We used Laser Capture Microdisection to isolate RNA from ATZ-globule-free hepatocytes and ATZ-globule-containing hepatocytes and subjected them to microarray analysis to detail the differential global gene expression profiles.

2017-08-25 | GSE84763 | GEO

RNA-seq in livers of wild-type (WT) controls, PiZ and PIZ mice deleted for Chop (PiZ/Chop-/-)

Project description:Transgenic PiZ mice have been genetically engineered to express ATZ and have been a valuable experimental model for liver disease due to AAT polymerization. ATZ accumulates in these mice within the endoplamic reticulum (ER) of hepatocytes in a nearly identical manner to livers of affected patients. To investigate the role of the transcription factor CHOP in the pathogenesis of liver damage induced by ATZ, we performed RNA-seq in livers of 6-week-old wild type, PiZ and PiZ mice deleted for Chop. All groups were matched for the strain, age, and the gender.

2020-12-01 | GSE118290 | GEO

Gene expression analysis of PiZ mouse livers

Project description:Transgenic PiZ mice have been genetically engineered to express ATZ and have been a valuable experimental model for studing liver disease associated with AAT deficiency. ATZ accumulates in these mice within the ER of hepatocytes in a nearly identical manner to livers of affected patients. To investigate the pathogenesis of liver damage induced by ATZ, we performed gene expression analysis in livers of 6-week-old PiZ mice and strain-, age-, and gender-matched wild-type mouse controls. All samples were processed on Affymetrix Mouse 430A 2.0 arrays using GeneChip 3’-IVT Plus and Hybridization Wash and Stain kits by means of Affymetrix’s standard protocols. The analysis indicated that most genes upregulated in PiZ livers were associated with response to unfolded proteins, ER nuclear signaling pathway, and response to protein stimulus.

2017-03-23 | GSE93115 | GEO

Liver miRNA profile of PiZ mice

Project description:Transgenic PiZ mice have been genetically engineered to express ATZ and have been a valuable experimental model for liver disease due to AAT polymerization. ATZ accumulates in these mice within the ER of hepatocytes in a nearly identical manner to livers of affected patients. MiRNAs play a key role in a wide range of biological processes and regulate gene expression mainly at the translational level. To search for miRNA with altered expression in AAT liver disease, we analyzed by miRNA next generation sequencing (NGS) the livers of 6-week-old PiZ mice and strain-, age-, and gender-matched wild-type mouse controls. This analysis revealed 70 miRNAs with differential expressions.

2017-03-23 | GSE85413 | GEO

RNA-seq in livers of wild-type (WT) controls, PiZ and PIZ mice deleted for Chop (PiZ/Chop-/-)

Project description:RNA-seq in livers of wild-type (WT) controls, PiZ and PIZ mice deleted for Chop (PiZ/Chop-/-)

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Comprehensive characterization of hepatic inflammatory changes in a mouse model of alpha-1 antitrypsin deficiency

Project description:In this study, we sought to thoroughly characterize the liver pathophysiology of a human transgenic mouse model for alpha-1 antitrypsin deficiency (AATD) with a strong manifestation of AATD-mediated liver disease. Male and female transgenic mice for normal variant human alpha-1 antitrypsin (Pi*M) and mutant human alpha-1 antitrypsin (Pi*Z) at 3 and 6 months of age with a C57BL/6J background were subjected to study. The progression of hepatic accumulation of mutant alpha-1 antitrypsin (ZAAT), hepatocyte injury, steatosis, liver inflammation and fibrotic features of this mouse model were monitored by performing an in vivo study.

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Transcriptomic analyses of the effects of hepatic insulin signaling on hepatic gene expression in the PiZ mouse model of alpha-1-antitrypsin deficiency liver disease

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2022-07-05 | GSE179416 | GEO

HRD1-mediated METTL14 degradation regulates m6A mRNA modification to suppress ER proteotoxic liver disease

Project description:Accumulation of unfolded or misfolded proteins in the endoplasmic reticulum (ER) lumen triggers unfolded protein response (UPR) for stress adaptation, the failure of which induces cell apoptosis and tissue/organ damage. The molecular switches underlying how the UPR selects for stress adaptation over apoptosis remain unknown. Here we discovered that accumulation of unfolded/misfolded proteins selectively induces N6-adenosine-methyltransferase-14 (METTL14) expression. METTL14 promotes CHOP mRNA decay through its 3’UTR N6-adenosine methylation (m6A) to inhibit its downstream pro-apoptotic target genes expression. UPR induces METTL14 expression through competing the HRD1-ERAD machinery to block METTL14 ubiquitination and degradation. Therefore, mice with liver-specific METTL14 deletion are highly susceptible to both acute pharmacological and alpha-1 antitrypsin (AAT) deficiency-induced ER proteotoxic stress and liver injury. Further hepatic CHOP deletion protects METTL14 knockout mice from ER stress-induced liver damage. Our study reveals a crosstalk between ER stress and mRNA m6A pathways, the ERm6A pathway, for ER stress adaptation to proteotoxicity.

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