Dataset Information

Lmnadelta9 mouse gene expression study

ABSTRACT: Lmnadelta9 mice derived in the C57bl6X129S3/J background have been identified as exhibiting a progeric phenotype. Using this mouse model we show that a truncated variant of Lamin A (Lmna∆9) causes the proliferative arrest of post-natal fibroblasts. Arrest is due to the cells inability to produce a functional extracellular matrix (ECM). We used microarrays to compare gene expression of adult primary fibroblasts from wildtype and Lmnadelta9 mice in order to understand whether the Lmnadelta9 adult MEF defective proliferation is due to alterations of specific signaling associated with ECM functions.

ORGANISM(S): Mus musculus

PROVIDER: GSE23495 | GEO | 2010/08/09

SECONDARY ACCESSION(S): PRJNA131003

REPOSITORIES: GEO

ACCESS DATA

Dataset's files

Source:

			Action	DRS
		Other

Items per page:

1 - 1 of 1

Similar Datasets

Project description:Background: Lamins A/C (encoded by the LMNA gene) can lead to dilated cardiomyopathy (DCM). Objectives: This study sought to undertake proteomic analysis of myocardial tissue to explore the postgenomic phenotype of end-stage lamin heart disease. Methods: Consecutive patients with end-stage lamin heart disease (LMNA-group, n=7) and ischaemic DCM (ICM-group, n=7) undergoing heart transplantation were enrolled. Samples were obtained from left atrium(LA), left ventricle(LV), right atrium(RA), right ventricle(RV) and interventricular septum(IVS). Liquid chromatography combined with mass-spectrometry was used for protein quantification. We compared protein concentrations in cardiac samples between LMNA and ICM groups. Proteins were considered differentially abundant if the quantitative difference was 1.5-fold and corrected p-value <0.05 at a false discovery rate of 0.01. Gene ontology(GO) enrichment analysis explored the related biological processes. Results: 4,247 proteins were identified in LMNA and ICM samples, of which 633 were differentially abundant in LA, 39 in LV, 181 in RA, 52 in RV, and 85 in IVS. Abundance of lamin A/C was reduced but lamin B (LMNB) increased in LMNA LA/RA tissue compared to ICM, but not in LV/RV. Transthyretin was more abundant in the LV/RV of LMNA compared to ICM while sarcomeric proteins such as titin and cardiac myosin heavy chain were generally reduced in RA/LA of LMNA. Protein expression profiling and GO enrichment analysis revealed sarcopenia, extracellular matrix(ECM) remodeling, deficient myocardial energetics, redox imbalances, and abnormal calcium handling in LMNA samples. Conclusion: Lamin heart disease is a biventricular and biatrial disease, characterized by sarcopenia, aberrant metabolism, and ECM remodeling. LMNB and transthyretin were unexpectedly abundant in the atria and ventricles respectively of patients with end-stage lamin heart disease potentially hinting to the possibility of compensatory responses.

Dataset Information

Lmnadelta9 mouse gene expression study

Dataset's files

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets