An evaluation of CaP in 16 mutation carriers and in 12 first-degree relatives from 288 Danish LS families found that tumors with mutations in MSH2, MLH1, and MSH6 genes had higher Gleason grades, displayed MSI-high phenotype and loss of the respective MMR proteins in a subset of the tumors, further demonstrating the link between LS and CaP [105]. Here, MSH2 is linked to Leigh syndrome.