Although the immune signature in premalignant lesions in LS described above by Chang et al. was determined to be independent of mutational rate and neoantigen load, the upregulation of the regulatory T cell–related gene FOXP3 and corresponding FOXP3+ T cell infiltration was specifically observed in dMMR hypermutated LS polyps (Fig. 5) [66]. Here, FOXP3 is linked to Leigh syndrome.