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Status |
Public on Mar 01, 2009 |
Title |
Gene expression profiling of Galgt2 overexpression in mdx skeletal muscle |
Organism |
Mus musculus |
Experiment type |
Expression profiling by array
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Summary |
Transgenic overexpression of Galgt2 in the skeletal muscles of mdx mice inhibits the development of disease pathology associated with muscular dystrophy. This is the case both in transgenic mice, where Galgt2 overexpression occurs from embryonic timepoints onward and in mdx mice where Galgt2 is overexpressed in the early postnatal period using Adeno-associated virus (AAV). Here, we use gene expression profiling to compare transcriptional changes resulting from embryonic and postnatal Galgt2 overexpression in mdx skeletal muscle. A surprising number of changes were in genes known to ameliorate muscular dystrophy when overexpressed (agrin, integrin alpha 7, ADAM12, Bcl2) or to cause muscular dystrophy when mutated (collagen VI (alpha1,alpha2), plectin 1, dystroglycan, selenoprotein N1, integrin alpha7, biglycan, dysferlin). Several genes involved in calcium homeostasis were also changed. In Galgt2 transgenic mice, where embryonic overexpression of Galgt2 in skeletal muscles alters neuromuscular development and muscle growth, the number of gene expression changes was vastly greater, however, 14% of genes altered in postnatal AAV-Galgt2 infected mdx muscles were also changed with embryonic overexpression. These experiments suggest that postnatal overexpression of Galgt2 inhibits muscular dystrophy in mdx mice via induction of a group of genes that, in aggregate, can govern membrane stability, membrane repair, calcium homeostasis, and apoptosis. Keywords: disease state analysis, gene therapy, comparative gene expression, muscular dystrophy, glycosylation, collagens, integrins, dysferlin, dystroglycan
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Overall design |
Microarrays were done in three groups at a time. 1) Transgenic Overexpression of Galgt2 - comprised to Wild Type (WT), Galgt2 Transgenic (CT), Dystrophin-Deficient (mdx), and Galgt2 Transgenic and Dystrophin-Deficient (CTmdx) each in duplicate 2) AAV-Mediated Galgt2 Gene Delivery in to the mdx gastrocnemius muscle in the postnatal period - comprised of AAVGalgt2 and PBS each in duplicate, and 3) Myoblasts and myotubes - comptised of one sample each of C1C12 myoblasts and myotubes, C2C12 myoblasts and myotubes stably transfected with Galgt2
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Contributor(s) |
Chandrasekharan K, Martin PT |
Citation missing |
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Submission date |
May 21, 2007 |
Last update date |
Feb 11, 2019 |
Contact name |
Paul T Martin |
E-mail(s) |
MartinPT@pediatrics.ohio-state.edu
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Organization name |
Columbus Children's Research Institute
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Street address |
700 Children's Drive
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City |
Columbus |
State/province |
OH |
ZIP/Postal code |
43205 |
Country |
USA |
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Platforms (1) |
GPL1261 |
[Mouse430_2] Affymetrix Mouse Genome 430 2.0 Array |
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Samples (16)
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GSM190638 |
Gastrocnemius Muscle mdx rep2 |
GSM190639 |
Gastrocnemius Muscle Galgt2 Transgenic rep1 |
GSM190640 |
Gastrocnemius Muscle Galgt2 Transgenic rep2 |
GSM190641 |
Gastrocnemius Muscle Galgt2 Transgenic mdx rep1 |
GSM190642 |
Gastrocnemius Muscle Galgt2 Transgenic mdx rep2 |
GSM190643 |
Gastrocnemius Muscle mdx AAV-Galgt2-Treated rep1 |
GSM190644 |
Gastrocnemius Muscle mdx AAV-Galgt2-Treated rep2 |
GSM190645 |
Gastrocnemius Muscle mdx PBS-Treated rep1 |
GSM190646 |
Gastrocnemius Muscle mdx PBS-Treated rep2 |
GSM190647 |
Myoblast Cell Line rep1 |
GSM190648 |
Myoblast Cell Line Differentiated rep1 |
GSM190649 |
Myoblast Cell Line Stably Transfected with Galgt2 rep1 |
GSM190650 |
Myoblast Cell Line Stably Transfected with Galgt2 Differentiated rep1 |
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Relations |
BioProject |
PRJNA100003 |
Supplementary file |
Size |
Download |
File type/resource |
GSE7863_RAW.tar |
60.2 Mb |
(http)(custom) |
TAR (of CEL) |
Processed data included within Sample table |
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