The SOU is constituted of mitochondrial membrane-bound sulfide quinone reductase (SQR) and two other enzymes the sulfur dioxygenase (ETHE1, also called dioxygenase ethylmalonic encephalopathy) and the thiosulfate sulfurtransferase (TST, also known as one isoenzyme of the rhodanese), ensuring the final oxidation of the two disulfides consuming molecular oxygen and water [68]. This evidence concerns the gene ETHE1 and Encephalopathy.