Table 1.

Examples of mitochondrial enzyme dysfunction in cancer development.

Genes	Main Features and Mechanism	Cancer Types	References
IDH1/2 mutations	Accumulation of oncometabolites D-2HG, epigenetic alterations, and impaired mitochondrial respiration	Gliomas, glioblastomas, and AML	[11,12,13,14,15,16,17,18,19,20]
SDH mutations	Accumulation of succinate, inhibition of PHDs, and HIF1a stabilization	Pheochromocytoma, paraganglioma, and other cancers	[21,22,23,24,25,26,27,28,29,30,31,32,33,34]
FH mutations	Abnormal accumulation of fumarate, inhibition of PHDs, and HIF1a stabilization. Glutamine is used for heme synthesis. Glutamine is converted to aKG for citrate formation. Glutamine is also used to fuel TCA cycle and ATP production	Papillary renal cell cancer, leiomyosarcoma, pheochromocytoma, paragangliomas, and other cancers	[35,36,37,38,39,40,41,42,43,44,45,46]
SOD1-/-	Accumulation of O2-, abnormal mitochondria, oxidation of DNA, protein, and lipid, and decreased aconitase activity	Hepatocellular carcinoma	[47]
SOD2+/-	Mitochondrial oxidative damage, nuclear DNA/ mtDNA oxidation, and decreased mitochondrial membrane potential	Lymphoma, pituitary adenoma	[48]