An iron protein. Glutathione (GSH) plays a catalytic role in elemental sulfur activation, but is not consumed during the enzymic reaction. GSH and elemental sulfur react non-enzymically to yield S-sulfanylglutathione and it is only in this form that the sulfur can be oxidized to yield sulfite. The sulfite can be further converted into sulfate, thiosulfate or S-sulfoglutathione (GSSO3-) non-enzymically .
ethylmalonic encephalopathy is caused by mutations in the mitochondrial matrix sulfur dioxygenase ETHE1 leading to failure to detoxify sulfide, a product of intestinal anaerobes and, in trace amounts, tissues
the combined oral treatment with metronidazole and N-acetylcysteine is an effective therapy for ethylmalonic encephalopathy which is caused by mutations in the mitochondrial matrix sulfur dioxygenase ETHE1