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| Status |
Public on Dec 02, 2006 |
| Title |
Comparison between gene expression in heart from Lmna H222P heterozygous and control mice |
| Organism |
Mus musculus |
| Experiment type |
Expression profiling by array
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| 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
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| 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.
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| 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 |
| E-mail(s) |
am2434@columbia.edu
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| Phone |
212 305 4088
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| Organization name |
columbia university
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| Department |
Medicine
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| Street address |
630W 168th street
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| City |
New York |
| State/province |
NY |
| ZIP/Postal code |
10032 |
| Country |
USA |
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| Platforms (1) |
| GPL1261 |
[Mouse430_2] Affymetrix Mouse Genome 430 2.0 Array |
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| Samples (15)
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| This SubSeries is part of SuperSeries: |
| GSE8000 |
Activation of MAPK pathways links LMNA mutations to cardiomyopathy in Emery-Dreifuss muscular dystrophy |
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| Relations |
| BioProject |
PRJNA105003 |
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