This highlights a recurring theme amongst hereditary RCC of mitochondrial oncometabolite accumulation which causes various mitochondrial and cellular PTMs (i.e., acetylation of lysine residues in VHL lesions; succinylation of lysine residues with SDH-RCC and succination of cysteine residues in HLRCC) and thus promotes tumorigenesis. This evidence concerns the gene SDHB and renal cell carcinoma.