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Status |
Public on Jul 16, 2003 |
Title |
Gene expression profiling in DQA1*0501+ children with untreated dermatomyositis |
Organism |
Homo sapiens |
Experiment type |
Expression profiling by array
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Summary |
Juvenile dermatomyositis (JDM), the most common pediatric inflammatory myopathy, is a systemic vasculopathy affecting young children. Epidemiology studies documenting an antecedent illness in the 3 mo before the first definite symptom (rash and/or weakness) of JDM are supported by immunologic data that suggest that the disease pathophysiology is Ag driven. The purpose of this study was to compare the gene expression profiles in muscle biopsies of four untreated DQA1*0501(+) JDM children with profiles from children with a known necrotizing myopathy (Duchenne muscular dystrophy), as well as an in vitro antiviral model (NF90), and healthy pediatric controls. Nearly half (47%) of the dysregulated genes in JDM were associated with the immune response. In particular, increased expression of IFN-alphabeta-inducible genes 6-16, myxovirus resistance protein p78, latent cytosolic transcription factor, LMP2, and TAP1 was observed. This profile is consistent with an IFN-alphabeta transcription cascade seen in the in vitro viral resistance model. The IFN-alphabeta-inducible profile was superimposed on transcription profiles reflective of myofiber necrosis and regeneration shared with Duchenne muscular dystrophy. Expressed genes were confirmed by quantitative real-time PCR (6-16), immunofluorescence (thrombospondin 4), and immunolocalization (IFN-gamma, p21). Keywords: other
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Contributor(s) |
Tezak Z, Hoffman EP, Lutz JL, Fedczyna TO, Stephan D, Bremer EG, Krasnoselska-Riz I, Kumar A, Pachman LM |
Citation(s) |
11937576 |
Submission date |
Jul 01, 2003 |
Last update date |
Oct 01, 2019 |
Contact name |
Eric P Hoffman |
E-mail(s) |
ericphoffman@gmail.com
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Organization name |
Binghamton University - SUNY
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Department |
Pharmaceutical Sciences
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Street address |
PO Box 6000
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City |
Binghamton |
State/province |
NY |
ZIP/Postal code |
13902 |
Country |
USA |
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Platforms (2) |
GPL80 |
[Hu6800] Affymetrix Human Full Length HuGeneFL Array |
GPL246 |
Affymetrix GeneChip Custom-Made Muscle Chip |
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Samples (14)
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Relations |
BioProject |
PRJNA85195 |
Supplementary file |
Size |
Download |
File type/resource |
GSE493_RAW.tar |
29.0 Mb |
(http)(custom) |
TAR (of CEL) |
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