Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
10219253 | Translational Research | 2018 | 30 Pages |
Abstract
The present review aims to summarize available knowledge on the role of the ubiquitin-proteasome system (UPS) in the pathogenesis of scleroderma and scleroderma-related disease mechanisms. This will provide the reader with a more mechanistic understanding of disease pathogenesis and help to identify putative novel targets within the UPS for potential therapeutic intervention. Because of the heterogenous manifestations of scleroderma, we will primarily focus on conserved mechanisms that are involved in the development of lung scleroderma phenotypes.
Keywords
TLRTNFPRRsGM-CSFECMTGFβSSCILDAdenosine TriphosphateATPsystemic sclerosisinterferonIFNinterleukinInterstitial lung diseaseTransforming growth factor βToll-like receptorgranulocyte-macrophage colony-stimulating factortumor necrosis factorExtracellular matrixmajor histocompatibility complexMHCpattern recognition receptors
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Authors
Silke Meiners, John Evankovich, Rama K. Mallampalli,