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Additional Supporting Information may be found in the online version of this article.

FilenameFormatSizeDescription
ART_33368_sm_SupplTable1.doc32KSupplementary Table 1
ART_33368_sm_SupplTable2.pdf72KSupplementary Table 2
ART_33368_sm_SupplFig1.tif28KSupplementary Figure 1. Model of dysregulated IFN pathways in CANDLE. Mutant i-proteasome induced IFN signaling abnormality is depicted in this tentative model. In response to a trigger such as infections, IFNs are produced which upregulate the expression of the i-proteasome to process the accumulating polyubiquitinated proteins. Defects in i-proteasomes caused by PSMB8 mutations results in increase of polyubiquitinated proteins and cell stress, which then trigger more IFN production, leading to a vicious cycle.
ART_33368_sm_SupplFig2.tif1483KSupplementary Figure 2. IFN signature under treatment with biologics in one CANDLE patient. Various treatments with biologics [TNF-alpha inhibition (adalimumab), IL-6 inhibition (tocilizumab), inhibition of the CD28/CD86 interaction (abatacept), or a calcineurin inhibitor (tacrolimus)] led to persistence of the interferon signature on microarray analysis. In red are upregulated genes compared to healthy age matched children.

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