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| Status |
Public on Mar 19, 2015 |
| Title |
Expression data from regenerating mouse TA muscle |
| Organism |
Mus musculus |
| Experiment type |
Expression profiling by array
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| Summary |
Mutations in fukutin-related protein (FKRP) gene are characterized with a lack of functionally glycosylated α-dystroglycan (F-α-DG). Surprisingly a small number of muscle fibers express strong F-α-DG. Here we investigate restoration of F-α-DG in FKRP mutant muscle and showed that the restoration of glycosylation is associated with muscle regeneration and dependent of the expression of both LARGE and partially functional FKRP. F-α-DG in regenerating fibers reaches normal levels and lasts for more than 4 weeks, however, no up-regulation of the LARGE and FKRP is detected during the regeneration process. These results suggest that FKRP P448L mutation clinically associated with severe CMD retains at least partial functions. Identification of factor(s) other than LARGE and FKRP could create new approaches for restoration of F-α-DG in mature muscle fibers with defects in FKRP function.
We employed microarrays to detail changes in gene expression associated with muscle regeneration in wild-type and FKRP mutant skeletal muscle, and have identified a group of genes with altered expression between these mice during this regeneration process.
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| Overall design |
RNA from TA muscles of untreated control and i.m. NTX treated FKRP (P448L) mutant mice were compared by microarray at 2, 4, and 14 days post treatment to assess regenerative effects on gene expression.
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| Contributor(s) |
Lu QL, Awano H, Tucker JD |
| Citation(s) |
25976249 |
| Submission date |
Mar 18, 2015 |
| Last update date |
Aug 06, 2018 |
| Contact name |
Qi Long Lu |
| Organization name |
Carolinas HealthCare System
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| Department |
Neurology
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| Lab |
McColl Lockwood Laboratory for Muscular Dystrophy Research
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| Street address |
1000 Blythe Blvd
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| City |
Charlotte |
| State/province |
North Carolina |
| ZIP/Postal code |
28203 |
| Country |
USA |
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| Platforms (1) |
| GPL11180 |
[HT_MG-430_PM] Affymetrix HT MG-430 PM Array Plate |
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| Samples (24)
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| GSM1636473 |
WT, Untreated, biological rep1 C57.Untr_1 |
| GSM1636474 |
WT, Untreated, biological rep2 C57.Untr_2 |
| GSM1636475 |
WT, Untreated, biological rep3 C57.Untr_3 |
| GSM1636476 |
Mut, Untreated, biological rep1 Mut.Untr_1 |
| GSM1636477 |
Mut, Untreated, biological rep2 Mut.Untr_2 |
| GSM1636478 |
Mut, Untreated, biological rep3 Mut.Untr_3 |
| GSM1636479 |
WT, NTX Treated, biological rep1 C57.NTX_1 |
| GSM1636480 |
WT, NTX Treated, biological rep2 C57.NTX_2 |
| GSM1636481 |
WT, NTX Treated, biological rep3 C57.NTX_3 |
| GSM1636482 |
Mut, NTX Treated, biological rep1 Mut.NTX.4_1 |
| GSM1636483 |
Mut, NTX Treated, biological rep2 Mut.NTX.4_2 |
| GSM1636484 |
Mut, NTX Treated, biological rep3 Mut.NTX.4_3 |
| GSM1636485 |
Mut, Untreated, biological rep4 Mut.Untr_1 |
| GSM1636486 |
Mut, NTX Treated, biological rep1 Mut.NTX.2_1 |
| GSM1636487 |
Mut, NTX Treated, biological rep2 Mut.NTX.2_2 |
| GSM1636488 |
Mut, NTX Treated, biological rep3 Mut.NTX.2_3 |
| GSM1636489 |
Mut, NTX Treated, biological rep4 Mut.NTX.2_4 |
| GSM1636490 |
Mut, NTX Treated, biological rep1 Mut.NTX.14_1 |
| GSM1636491 |
Mut, NTX Treated, biological rep2 Mut.NTX.14_2 |
| GSM1636492 |
Mut, NTX Treated, biological rep3 Mut.NTX.14_3 |
| GSM1636493 |
Mut, PEI Treated, biological rep1 Mut.PEI.2_1 |
| GSM1636494 |
Mut, PEI Treated, biological rep2 Mut.PEI.2_2 |
| GSM1636495 |
Mut, PEI Treated, biological rep3 Mut.PEI.2_3 |
| GSM1636496 |
Mut, PEI Treated, biological rep4 Mut.PEI.2_4 |
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| Relations |
| BioProject |
PRJNA278754 |
| Supplementary file |
Size |
Download |
File type/resource |
| GSE67032_RAW.tar |
49.0 Mb |
(http)(custom) |
TAR (of CEL) |
| GSE67032_nd14_vs_cm.txt.gz |
2.0 Kb |
(ftp)(http) |
TXT |
| GSE67032_nd2_vs_cm.txt.gz |
168.6 Kb |
(ftp)(http) |
TXT |
| GSE67032_nd4_vs_cm.txt.gz |
71.3 Kb |
(ftp)(http) |
TXT |
| Processed data included within Sample table |
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