NLRP3 and Schwartz-Jampel syndrome: In our experiments, the results show that the oxygen consumption rate is significantly reduced in patients with SJS/TEN as compared to the normal control which is expected as the mitochondria is damaged in SJS/TEN, leading to more mtROS accumulation which causes a higher activation of the NLRP3 inflammasome in these patients along with a reduced oxygen consumption rate in their cells.