ETHE1 is a sulfur dioxygenase that localizes within the mitochondrial matrix. The enzyme functions in sulfide catabolism. Mutations in its gene result in ethylmalonic encephalopathy.
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Protein Aliases: ethylmalonic encephalopathy 1; Ethylmalonic encephalopathy protein 1; Ethylmalonic encephalopathy protein 1 homolog; Hepatoma subtracted clone one protein; HSCO; Persulfide dioxygenase ETHE1, mitochondrial; protein ETHE1, mitochondrial; Sulfur dioxygenase ETHE1
Gene Aliases: 0610025L15Rik; ETHE1; HSCO; YF13H12
Molecular Function: hydrolase