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Series GSE6398 Query DataSets for GSE6398
Status Public on Dec 02, 2006
Title Comparison between gene expression in heart from Lmna H222P homozygous and control mice
Organism Mus musculus
Experiment type Expression profiling by array
Summary 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
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.
Contributor(s) Muchir a, Pavlidis p, Decostre v, Herron A, Arimura t, Bonne g, Worman hj
Citation(s) 17446932
Submission date Nov 29, 2006
Last update date Feb 11, 2019
Contact name antoine Muchir
Phone 212 305 4088
Organization name columbia university
Department Medicine
Street address 630W 168th street
City New York
State/province NY
ZIP/Postal code 10032
Country USA
Platforms (1)
GPL1261 [Mouse430_2] Affymetrix Mouse Genome 430 2.0 Array
Samples (14)
GSM147451 Control male 1
GSM147452 Control male 2
GSM147459 Control male 3
This SubSeries is part of SuperSeries:
GSE8000 Activation of MAPK pathways links LMNA mutations to cardiomyopathy in Emery-Dreifuss muscular dystrophy
BioProject PRJNA105005

Download family Format
SOFT formatted family file(s) SOFTHelp
MINiML formatted family file(s) MINiMLHelp
Series Matrix File(s) TXTHelp

Supplementary file Size Download File type/resource
GSE6398_RAW.tar 84.8 Mb (http)(custom) TAR (of CEL)
Raw data provided as supplementary file

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