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| Status |
Public on May 18, 2011 |
| Title |
Neurofibromin (Nf1) is required for skeletal muscle development |
| Organism |
Mus musculus |
| Experiment type |
Expression profiling by array
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| Summary |
Neurofibromatosis type 1 (NF1) is a multi-system disease caused by mutations in the NF1 gene encoding a Ras-GAP protein, neurofibromin, which negatively regulates Ras signalling. Besides neuroectodermal malformations and tumours, the skeletal system is often affected (e.g. scoliosis and long bone dysplasia), demonstrating the importance of neurofibromin for development and maintenance of the musculoskeletal system. Here we focus on the role of neurofibromin in skeletal muscle development. Nf1 gene inactivation in the early limb bud mesenchyme using Prx1-cre (Nf1Prx1) resulted in muscle dystrophy characterised by fibrosis, reduced number of muscle fibres, and reduced muscle force. To gain insight into the molecular changes of the observed muscle dystrophy and fibrosis and to compare these with other known muscle dystrophies, we performed transcriptional profiling of the entire triceps muscles of threemonth-old wild type (wt) and mutant animals using Affymetrix high-density microrrays.
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| Overall design |
We analyzed triceps muscles from 4 three-month-old wt controls and 4 three-month-old Nf1Prx1 mice using the Affymetrix Mouse Gene 1.0 ST platform. Array data was processed by the Affymetrix Exon Array Computational Tool. RNA isolated from each animal was hybridized to a separate microarray.
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| Contributor(s) |
Kossler N, Stricker S, Rödelsperger C, Robinson PN, Kim J, Dietrich C, Osswald M, Kühnisch J, Stevenson DA, Braun T, Mundlos S, Kolanczyk M |
| Citation(s) |
21478499 |
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| Submission date |
May 17, 2011 |
| Last update date |
Mar 04, 2019 |
| Contact name |
Mateusz Kolanczyk |
| E-mail(s) |
[email protected]
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| Organization name |
Max Planck Institute
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| Street address |
Ihnestr 73
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| City |
Berlin |
| ZIP/Postal code |
14195 |
| Country |
Germany |
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| Platforms (1) |
| GPL6246 |
[MoGene-1_0-st] Affymetrix Mouse Gene 1.0 ST Array [transcript (gene) version] |
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| Samples (8)
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| Relations |
| BioProject |
PRJNA140153 |
| Supplementary file |
Size |
Download |
File type/resource |
| GSE29343_2010e19_10Exp_musc_ctrl_vs_mut.txt.gz |
15.3 Mb |
(ftp)(http) |
TXT |
| GSE29343_RAW.tar |
36.2 Mb |
(http)(custom) |
TAR (of CEL, CHP) |
| GSE29343_musc_ctrl_vs_mut_do.txt.gz |
106.0 Kb |
(ftp)(http) |
TXT |
| GSE29343_musc_ctrl_vs_mut_up.txt.gz |
165.1 Kb |
(ftp)(http) |
TXT |
| GSE29343_readme.txt |
2.3 Kb |
(ftp)(http) |
TXT |
| Processed data included within Sample table |
| Processed data provided as supplementary file |
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