- Mitochondrial acetoacetyl-CoA thiolase (T2) deficiency: T2-deficient patients with "mild" mutation(s) were previously misinterpreted as normal by the coupled assay with tiglyl-CoA. Zhang, G.X., Fukao, T., Rolland, M.O., Zabot, M.T., Renom, G., Touma, E., Kondo, M., Matsuo, N., Kondo, N. Pediatr. Res. (2004)