Elucidation of the pathogenesis of somatic mosaic CINCA syndrome. (A) Cytokine secretion from iPS-MPs derived from patient 1. After stimulating iPS-MPs by LPS with or without ATP, we determined the IL-1β (top panel), IL-6 (middle panel), or TNFα (bottom panel) level of the supernatant. n = 3. (B) Cytokine secretion from iPS-MPs derived from patient 2, determined as in panel A. (C) IL-1β secretion from mutant iPS-MPs in the presence of 10-fold dilutions of LPS from 100 ng/mL. n = 3. (D) IL-1β secretion from wild-type iPS-MPs, determined as in panel C. (E) LDH secretion from iPS-MPs stimulated with LPS in the presence or absence of the cathepsin B inhibitor, CA074Me. n = 3. Data are mean ± SEM. ***P < .001 (Student t test).