HERC4 and Leigh syndrome: To investigate the regulation of ubiquitination in the development of LSS, the top 10 (RNF67, HERC6, RNF218, SMURF1, NEDL2, WWP1, HERC4, SMURF2, RNF218, and RNF58) differentially expressed E3 ubiquitin ligase were obtained from GSE113212, and the expression of these 10 genes was assessed in HLF and LDH patients using qRT-PCR.