TLR4 and Barth syndrome: Furthermore, we have shown that unsaturated CLs regardless of their chain length are able to inhibit LPS-dependent inflammatory cytokine secretion and the activation of the alternative NLRP3 inflammasome pathway (Figs. 2 and 7), while cardiolipins with saturated chains longer than 14 carbon atoms, like those found in patients with BTHS and in some bacteria [2, 38, 39], lose their anti-inflammatory properties (Fig. 2), and are instead able to mimic LPS by activating TLR4 and inducing pro-inflammatory cytokine secretion in macrophages and human blood-derived monocytes (Figs. 3, 4, and 5).