In addition to metabolic enzymes or transporters, accumulating “oncometabolites” due to defective TCA cycle enzymes, such as fumarate hydratase (FH), succinate dehydrogenase (SDH) and isocitrate dehydrogenase (IDH), were also detected in tumour cells implying that deregulated mitochondrial metabolites might also underlie oncogenic transformation [13, 14]. This evidence concerns the gene FH and neoplasm.