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Series GSE17914 Query DataSets for GSE17914
Status Public on Jun 30, 2011
Title IL13 and CCL2 Drive Disease in an Inflammatory Subset of Scleroderma [cGVHD]
Organism Mus musculus
Experiment type Expression profiling by array
Summary Scleroderma is a lethal and currently irreversible autoimmune disease characterized by widespread tissue fibrosis and vasculopathy. Using cross-species comparative gene expression profiling we show that murine sclerodematous graft-versus-host disease (sclGVHD) approximates an “inflammatory” subset of human scleroderma and that both diseases demonstrate activation of the IL13 cytokine pathway. We report that both host myeloid cells expressing type I and II activated macrophage markers and graft T-cells produce IL13 and that host mice deficient in either IL13 or IL4Rα, an IL13 signal transducer, are protected from disease. This signaling pathway converges on a single gene, CCL2, which is coordinately upregulated in sclGVHD, in the human inflammatory scleroderma subset and in IL13 treated human dermal fibroblasts. Accordingly, treatment with antibodies to CCL2, and its murine homolog CCL12, prevent sclGVHD. Lastly, we provide evidence that IL13 pathway activation in early scleroderma patients correlates with modified Rodnan skin scores (mRSS). These data indicate that an inflammatory subset of scleroderma is driven by IL13 and may benefit from IL13 or CCL2 blockade.
Overall design Sample vs reference. Total RNA isolated from skin of the following mice: B10->balb/c; balb/c->balb/c; B10->balb/c IL13-/-; balb/c->balb/c IL13-/-; B10->balb/c IL4Ra -/-; balb/c->balb/c IL4Ra -/-
Contributor(s) Sargent JL, Aliprantis AO, Greenblatt M, Farina G, Lafyatis R, Glimcher L, Whitfield ML, Tsang K
Citation(s) 22245215
Submission date Sep 01, 2009
Last update date Feb 18, 2020
Contact name Jennifer L Sargent
Organization name Dartmouth Medical School
Department Genetics
Lab Whitfield
Street address Dartmouth College; HB7400
City Hanover
State/province NH
ZIP/Postal code 03755
Country USA
Platforms (1)
GPL7202 Agilent-014868 Whole Mouse Genome Microarray 4x44K G4122F (Probe Name version)
Samples (28)
GSM447477 4533 B10.D2 -> balb rag2-/- IL4Ra/- (new)
GSM447478 4534 B10.D2 -> balb rag2-/- IL4Ra-/- (new)
GSM447479 4535 balb/c ->balb rag2-/- IL4Ra-/-(new)
This SubSeries is part of SuperSeries:
GSE24410 IL13 and CCL2 Drive Disease in an Inflammatory Subset of Scleroderma
BioProject PRJNA133067

Download family Format
SOFT formatted family file(s) SOFTHelp
MINiML formatted family file(s) MINiMLHelp
Series Matrix File(s) TXTHelp

Supplementary file Size Download File type/resource
GSE17914_RAW.tar 153.2 Mb (http)(custom) TAR (of GPR)
Processed data included within Sample table

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