In summary, this study is the first to show that the FDX1-LIPT1-DLAT ternary complex can exacerbate TCA cycle disorders by inhibiting DLAT activation, that the opening and closing of the mPTP can regulate cuproptosis, and that circKIAA1797 can regulate cuproptosis through these two pathways. Here, DLAT is linked to tricarboxylic acid cycle disorder.