Extended Data Fig. 2: Cytokine expression studies ex vivo in LUBAC-deficient patients.
From: Biallelic human SHARPIN loss of function induces autoinflammation and immunodeficiency
(a) Cytokine expression in LUBAC-deficient monocytes. PBMCs from SHARPIN (P1)- and HOIP-deficient patients and two healthy controls were stimulated with IL-1β (10 ng/ml) for 6 h, and the intracellular accumulation of cytokines in CD14+ monocytes was quantified by flow cytometry. (b, c) Cytokine secretion from LUBAC-deficient PBMCs. PBMCs from SHARPIN and HOIP-deficient patients and two healthy controls were stimulated with (b) LPS (1 μg/ml) or (c) IL-1β (10 ng/ml) for 6 h, and secreted cytokines were measured by ELISA. (d, e) Cytokine secretion from LUBAC-deficient fibroblasts. Fibroblasts from SHARPIN (P1)- and HOIP-deficient patients and two healthy controls were stimulated with (d) LPS (1 μg/ml) or (e) IL-1β (10 ng/ml) for 24 h, and secreted cytokines were measured by ELISA. (a-e) The experiments were performed with biological triplicates (a,d,e) or duplicates (b,c), and shown are the representatives of two independent experiments. Mean values ± s.d are displayed.
