In mammals, only four enzymatic complexes of lipoylated proteins have been identified, including those involved in the tricarboxylic acid cycle (PDH, alpha-ketoglutarate PDH, and KDH), branched-chain amino acid metabolism (BCKDH), and glycine metabolism (GCV)，when combined with Cu ions, lead to the accumulation of lipoylated proteins and mitochondrial metabolic dysfunction.
