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.

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.

Figure 2
fig2a.pptx - Immunoblot gel image underlying Figure 2a.
fig2b.pptx - Immunoblot gel image underlying Figure 2b.
fig2c MassSpec raw data - FXII peptides in gel.xlsx - Raw mass spectrometry data from excised gel fragments, underlying Figure 2c.
fig2d FXIIa Chromogenic.xlsx - Results of the chromogenic assay underlying Figure 2d.
fig2e FXIIa Chromogenic.xlsx - Results of the chromogenic assay underlying Figure 2e.

Figure 3
fig3a.pptx - Immunoblot gel underlying Figure 3a.
fig3c PK ELISA.xls - ELISA results measuring plasma prekallikrein, underlying Figure 3c.
fig3d,e enzyme-C1-INH complexes ELISA.xlsx - ELISA results measuring FXIIa-C1INH and PK-C1-INH complexes in plasma, underlying Figure 3d and 3e.
fig3f.pptx - Immunoblot gel image underlying Figure 3f.
fig3g cHMWK Chromogenic.xlsx - Results of the chromogenic assay measuring cHMWK activity, underlying Figure 3g.
fig3h BK ELISA.xls - ELISA results measuring bradykinin plasma levels, underlying Figure 3h.

Figure 4
fig4b count_analysis neutrophils.xlsx - Neutrophil counts from lesional skin of a FACAS patient, stained for myeloperoxidase and α-FXIIa heavy chain.
fig4b Image J example_FACAS_rgb.tif - Example ImageJ analysis, representative of Figure 4b.
fig4c IL-8 ELISA.xls - ELISA results measuring monocyte IL-8 secretion, underlying Figure 4c.

Figure 5
fig5a IL-1ß ELISA.xls - ELISA results underlying Figure 5a.
fig5b IL-1ß ELISA.xls - ELISA results underlying Figure 5b.
fig5c IL-1ß ELISA.xls - ELISA results underlying Figure 5c.

Supplementary Figures
figSupplementary1 FXII ELISA.xlsx - ELISA results comparing plasma FXII levels from FACAS plasma versus healthy control, underlying Supplementary Figure 1.
figSupplementary2.pptx - Immunoblot gel image underlying Supplementary Figure 2.
figSupplementary4 IL-1ß ELISA.xlsx - ELISA results underlying Supplementary Figure 4.