Figure source data for Cold-induced urticarial autoinflammatory syndrome related to factor XII activation

In the accompanying paper, an autosomal dominant disease characterized by cold-induced urticarial rash and systemic inflammation is described. The disease is associated with a mutation in the human coagulation Factor 12 gene (F12), and the term FXII-associated cold autoinflammatory syndrome (FACAS) is proposed to denote it. Functional studies show autocatalytic cleavage of FXII and profound activation of the contact system linked to IL-1ß-mediated inflammation.<br>The dataset presented here contains source data and images that underlie the main and supplementary figures included in the accompanying paper. Data are provided as a combination of Excel XLSX files, images with labelling prepared in PowerPoint (PPTX files) and a TIF image.<br><b>Figure 2</b><b>fig2a.pptx</b> - Immunoblot gel image underlying Figure 2a.<b>fig2b.pptx </b>- Immunoblot gel image underlying Figure 2b.<b>fig2c MassSpec raw data - FXII peptides in gel.xlsx</b> - Raw mass spectrometry data from excised gel fragments, underlying Figure 2c.<b>fig2d FXIIa Chromogenic.xlsx</b> - Results of the chromogenic assay underlying Figure 2d.<b>fig2e FXIIa Chromogenic.xlsx</b> - Results of the chromogenic assay underlying Figure 2e.<br><b>Figure 3</b><b>fig3a.pptx </b>- Immunoblot gel underlying Figure 3a.<b>fig3c PK ELISA.xls</b> - ELISA results measuring plasma prekallikrein, underlying Figure 3c.<b>fig3d,e enzyme-C1-INH complexes ELISA.xlsx</b> - ELISA results measuring FXIIa-C1INH and PK-C1-INH complexes in plasma, underlying Figure 3d and 3e.<b>fig3f.pptx</b> - Immunoblot gel image underlying Figure 3f.<b>fig3g cHMWK Chromogenic.xlsx</b> - Results of the chromogenic assay measuring cHMWK activity, underlying Figure 3g.<b>fig3h BK ELISA.xls</b> - ELISA results measuring bradykinin plasma levels, underlying Figure 3h.<br><b>Figure 4</b><b>fig4b count_analysis neutrophils.xlsx</b> - Neutrophil counts from lesional skin of a FACAS patient, stained for myeloperoxidase and α-FXIIa heavy chain.<b>fig4b Image J example_FACAS_rgb.tif </b>- Example ImageJ analysis, representative of Figure 4b.<b>fig4c IL-8 ELISA.xls</b> - ELISA results measuring monocyte IL-8 secretion, underlying Figure 4c.<br><b>Figure 5</b><b>fig5a IL-1ß ELISA.xls</b> - ELISA results underlying Figure 5a.<b>fig5b IL-1ß ELISA.xls</b> - ELISA results underlying Figure 5b.<b>fig5c IL-1ß ELISA.xls</b> - ELISA results underlying Figure 5c.<br><b>Supplementary Figures</b><b>figSupplementary1 FXII ELISA.xlsx </b>- ELISA results comparing plasma FXII levels from FACAS plasma versus healthy control, underlying Supplementary Figure 1.<b>figSupplementary2.pptx </b>- Immunoblot gel image underlying Supplementary Figure 2.<b>figSupplementary4 IL-1ß ELISA.xlsx</b> - ELISA results underlying Supplementary Figure 4.