IL13 and CCL2 Drive Disease in an Inflammatory Subset of Scleroderma [cGVHD]

ABSTRACT: 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 hum

ORGANISM(S): Mus musculus

SUBMITTER: Jennifer Sargent 

PROVIDER: E-GEOD-17914 | biostudies-arrayexpress |

REPOSITORIES: biostudies-arrayexpress