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| Status |
Public on Nov 21, 2012 |
| Title |
Transcriptional Alterations of Hamstring Muscle Contractures in Children with Cerebral Palsy |
| Organism |
Homo sapiens |
| Experiment type |
Expression profiling by array
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| Summary |
Cerebral palsy is primarily an upper motor neuron disease that results in a spectrum of progressive movement disorders. Secondary to the neurological lesion, muscles from patients with cerebral palsy are often spastic and form debilitating contractures that limit range of motion and joint function. With no genetic component, the pathology of skeletal muscle in cerebral palsy is a response to aberrant neurological input in ways that are not fully understood. This study was designed to gain further understanding of the skeletal muscle response to cerebral palsy using microarrays and correlating the transcriptional data with functional measures. Hamstring biopsies from gracilis and semitendinosus muscles were obtained from a cohort of patients with cerebral palsy (n=10) and typically developing patients (n=10) undergoing surgery. Affymetrix HG-U133A 2.0 chips (n=40) were used and expression data was verified for 6 transcripts using quantitative real-time PCR, as well as for two genes not on the microarray. Chips were clustered based on their expression and those from patients with cerebral palsy clustered separately. Significant genes were determined conservatively based on the overlap of three summarization algorithms (n=1,398). Significantly altered genes were analyzed for over-representation among gene ontologies, transcription factors, pathways, microRNA and muscle specific networks. These results centered on an increase in extracellular matrix expression in cerebral palsy as well as a decrease in metabolism and ubiquitin ligase activity. The increase in extracellular matrix products was correlated with mechanical measures demonstrating the importance in disability. These data lay a framework for further studies and novel therapies.
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| Overall design |
Skeletal muscle biopsies from both the gracilis and semitendinosus were obtained during surgery for 20 pediatric subjects for affymetrix microarray analysis. We obtained a group of 10 patients undergoing medial hamstring lengthening in the cerebral palsy group and 10 patients undergoing ACL reconstruction with hamstring autograft in the control group. This provided 40 microarrays in 4 groups to analyze the effect of cerebral palsy and also differences between muscles.
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| Contributor(s) |
Smith LR, Chambers HG, Subramaniam S, Lieber R |
| Citation(s) |
22956992 |
| Submission date |
Aug 05, 2011 |
| Last update date |
Dec 06, 2018 |
| Contact name |
Lucas Robert Smith |
| E-mail(s) |
lrs001@ucsd.edu
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| Organization name |
University of California San Diego
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| Department |
Bioengineering
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| Lab |
Muscle Physiology Laboratory
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| Street address |
3350 La Jolla Village Drive Mail Code 151
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| City |
San Diego |
| State/province |
CA |
| ZIP/Postal code |
92161 |
| Country |
USA |
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| Platforms (1) |
| GPL571 |
[HG-U133A_2] Affymetrix Human Genome U133A 2.0 Array |
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| Samples (40)
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| GSM774470 |
Typically Developing Control, Gracilis, Patient Replicate 1 |
| GSM774471 |
Typically Developing Control, Semitendinosus, Patient Replicate 1 |
| GSM774472 |
Typically Developing Control, Gracilis, Patient Replicate 2 |
| GSM774473 |
Typically Developing Control, Semitendinosus, Patient Replicate 2 |
| GSM774474 |
Typically Developing Control, Gracilis, Patient Replicate 3 |
| GSM774475 |
Typically Developing Control, Semitendinosus, Patient Replicate 3 |
| GSM774476 |
Typically Developing Control, Gracilis, Patient Replicate 4 |
| GSM774477 |
Typically Developing Control, Semitendinosus, Patient Replicate 4 |
| GSM774478 |
Typically Developing Control, Gracilis, Patient Replicate 5 |
| GSM774479 |
Typically Developing Control, Semitendinosus, Patient Replicate 5 |
| GSM774480 |
Typically Developing Control, Gracilis, Patient Replicate 6 |
| GSM774481 |
Typically Developing Control, Semitendinosus, Patient Replicate 6 |
| GSM774482 |
Typically Developing Control, Gracilis, Patient Replicate 7 |
| GSM774483 |
Typically Developing Control, Semitendinosus, Patient Replicate 7 |
| GSM774484 |
Typically Developing Control, Gracilis, Patient Replicate 8 |
| GSM774485 |
Typically Developing Control, Semitendinosus, Patient Replicate 8 |
| GSM774486 |
Typically Developing Control, Gracilis, Patient Replicate 9 |
| GSM774487 |
Typically Developing Control, Semitendinosus, Patient Replicate 9 |
| GSM774488 |
Typically Developing Control, Gracilis, Patient Replicate 10 |
| GSM774489 |
Typically Developing Control, Semitendinosus, Patient Replicate 10 |
| GSM774490 |
Cerebral Palsy, Gracilis, Patient Replicate 1 |
| GSM774491 |
Cerebral Palsy, Semitendinosus, Patient Replicate 1 |
| GSM774492 |
Cerebral Palsy, Gracilis, Patient Replicate 2 |
| GSM774493 |
Cerebral Palsy, Semitendinosus, Patient Replicate 2 |
| GSM774494 |
Cerebral Palsy, Gracilis, Patient Replicate 3 |
| GSM774495 |
Cerebral Palsy, Semitendinosus, Patient Replicate 3 |
| GSM774496 |
Cerebral Palsy, Gracilis, Patient Replicate 4 |
| GSM774497 |
Cerebral Palsy, Semitendinosus, Patient Replicate 4 |
| GSM774498 |
Cerebral Palsy, Gracilis, Patient Replicate 5 |
| GSM774499 |
Cerebral Palsy, Semitendinosus, Patient Replicate 5 |
| GSM774500 |
Cerebral Palsy, Gracilis, Patient Replicate 6 |
| GSM774501 |
Cerebral Palsy, Semitendinosus, Patient Replicate 6 |
| GSM774502 |
Cerebral Palsy, Gracilis, Patient Replicate 7 |
| GSM774503 |
Cerebral Palsy, Semitendinosus, Patient Replicate 7 |
| GSM774504 |
Cerebral Palsy, Gracilis, Patient Replicate 8 |
| GSM774505 |
Cerebral Palsy, Semitendinosus, Patient Replicate 8 |
| GSM774506 |
Cerebral Palsy, Gracilis, Patient Replicate 9 |
| GSM774507 |
Cerebral Palsy, Semitendinosus, Patient Replicate 9 |
| GSM774508 |
Cerebral Palsy, Gracilis, Patient Replicate 10 |
| GSM774509 |
Cerebral Palsy, Semitendinosus, Patient Replicate 10 |
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| Relations |
| BioProject |
PRJNA146049 |
| Supplementary file |
Size |
Download |
File type/resource |
| GSE31243_GCRMA.txt.gz |
3.9 Mb |
(ftp)(http) |
TXT |
| GSE31243_RAW.tar |
75.8 Mb |
(http)(custom) |
TAR (of CEL) |
| GSE31243_RMA.txt.gz |
3.9 Mb |
(ftp)(http) |
TXT |
| Processed data included within Sample table |
| Processed data are available on Series record |
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