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Comparison between gene expression in heart from Lmna H222P heterozygous and control mice

ABSTRACT: The present research is devoted to the identification of gene(s) severely affected by LMNA mutations, leading to striated muscle laminopathies and more specifically the cardiomyopathy. For this purpose, we developped a large-scale gene expression approach on heart and skeletal tissues from Lmna H222P heterozygous Knock-In mouse model. Keywords: disease state modification

ORGANISM(S): Mus musculus

PROVIDER: GSE6397 | GEO | 2006/12/02

SECONDARY ACCESSION(S): PRJNA105003

REPOSITORIES: GEO

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Comparison between gene expression in heart from Lmna H222P homozygous and control mice

2006-12-02 | GSE6398 | GEO

Transcription profiling of mouse heart from Lmna H222P heterozygous and control mice

Project description:The present research is devoted to the identification of gene(s) severely affected by LMNA mutations, leading to striated muscle laminopathies and more specifically the cardiomyopathy. For this purpose, we developped a large-scale gene expression approach on heart and skeletal tissues from Lmna H222P heterozygous Knock-In mouse model. Experiment Overall Design: In the project presented here we performed differential expression in heart from a mouse model of EDMD: a LmnaH222P knock-in mouse created via homologous recombination by Gisele Bonne in Paris, France (Arimura et al., 2005). The mutant male LmnaH222P knock-in homozygous mice display reduced locomotion activity with abnormal stiff walking posture and all of them die by 9 months of age. As for cardiac phenotype, they develop chamber dilation and hypokinesia with conduction defects. These results demonstrate that LmnaH222P knock-in homozygous mice represents a good model for studying laminopathies affecting striated muscles as they develop a dystrophic condition of both skeletal and cardiac muscles reminiscent of the human diseases. Genes were identified as differentially expressed if they met a false discovery rate threshold of 0.05 in a two-sample t-test (q-value) and showed at least a two-fold difference in expression independent of absolute signal intensity.

2008-06-14 | E-GEOD-6397 | biostudies-arrayexpress

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transcriptomic data of soleus WT and Lmnap.H222P/H222P pre- and post-symptomatic mice

Project description:The present research is devoted to the identification of gene(s) severely affected by LMNA mutations, leading to striated muscle laminopathies and more specifically the skeletal phenotype of Emery-Freifuss Muscular Dystrophy. For this purpose, we developed a large-scale gene expression approach on soleus skeletal tissues from wild type Lmna H222P homozygous Knock-In mouse mode at pre and post stages of the disease .

2020-09-01 | GSE146112 | GEO

Transcription profiling of mouse heart from Emd KO and controls

Project description:The present research is devoted to the identification of gene(s) severely affected by EMD mutations, leading to striated muscle laminopathies and more specifically the cardiomyopathy. For this purpose, we developped a large-scale gene expression approach on heart and skeletal tissues from Emd KO mouse model. Experiment Overall Design: In the project presented here we performed differential expression in heart from a mouse model of EDMD: a Emd KO mouse created via homologous recombination by Y.Hayashi in Japan (Ozawa, 2006). The emd knock-out mouse showed significantly delayed PR time, that could correspond to impaired atrial-ventricle node conduction. Genes were identified as differentially expressed if they met a false discovery rate threshold of 0.05 in a two-sample t-test (q-value) and showed at least a two-fold difference in expression independent of absolute signal intensity.

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Functional and molecular analysis of LMNA mutations related muscular dystrophy in zebrafish

Project description:LMNA mutations cause laminopathies, a group of rare genetic diseases with no known cure, only symptomatic treatment and supportive care. In this study, we characterized five LMNA mutations (LMNA(L35P), LMNA(A539V), LMNA(W520G), LMNA(E358K), and LMNA(R453W)) identified from patients diagnosed with muscular laminopathy in the zebrafish model.

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Transcriptome analysis of C2C12 myoblasts with mutations in LMNA gene

Project description:Mutations in LMNA gene cause laminopathies in human. Mostly, laminopathies alter skeletal muscle tissue and lead to cardiomathy and lipodystrophy. We investigated the effect of mutations G232E (EDMD2 syndome) and R482L (FPLD2 syndrome) in LMNA gene on skeletal muclse functioning and metabolism using transgenic C2C12 myoblast cell lines and transcriptome analysis. We found abnormalities of nuclear lamina structure in mutant myoblasts, treir pro-myogenic commitment and metabolic disregulation on all stages of transgenic myoblasts differentiation.

2020-09-12 | GSE150365 | GEO

Transcription profiling of mouse heart samples from individuals with homozygous or heterozygous mutant alleles of Lamin A (LMNA)

Project description:This SuperSeries is composed of the following subset Series:; GSE6397: Comparison between gene expression in heart from Lmna H222P heterozygous and control mice; GSE6398: Comparison between gene expression in heart from Lmna H222P homozygous and control mice Experiment Overall Design: Refer to individual Series

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Gene expression profiles of livers from male LMNA+/+, LMNA flx/+, and LMNA flx/flx; Alb-Cre+ C57BL/6 mice fed normal chow or high fat diet

Project description:To investigate the molecular basis for male-specific steatohepatitis in Lamin A/C-deficient livers, microarray gene expression analysis was performed on total liver RNA isolated from 26- to 39-week-old, male LMNA+/+, LMNA flx/+, and LMNA flx/flx; Alb-Cre+ C57BL/6 mice fed either normal chow (NC) or high fat diet plus carbohydrate-supplemented water (HFD). Lamins are nuclear intermediate filament proteins that comprise the major components of the nuclear lamina in metazoan cells. Mutations in LMNA, which encodes lamins A/C, cause diseases termed laminopathies, including lipodystrophy, cardiomyopathy, and premature aging. The lamin A/C mutation-associated Dunnigan familial partial lipodystrophy is typically accompanied by fatty liver disease. The role of lamins in the liver is unknown, and it is unclear whether laminopathy-associated liver disease is due to primary hepatocyte defects or systemic alterations. To address these questions, mice carrying a hepatocyte-specific deletion of Lmna (KO mice) were generated. KO hepatocytes manifested abnormal nuclear morphology, and KO mice developed spontaneous male-selective hepatosteatosis, with increased susceptibility to high fat diet-induced steatohepatitis and fibrosis. The molecular mechanism by which liver-specific Lamin A/C deficiency induces male-specific steatohepatitis is unknown. The microarray data presented here demonstrates that hepatic Lmna deficiency is associated with upregulated expression of fatty acid transporters, lipid biosynthetic enzymes, lipid-droplet associated proteins, and interferon-regulated genes and other pro-inflammatory mediators.

2017-01-16 | GSE93643 | GEO

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