Protein: Persulfide dioxygenase ETHE1, mitochondrial (EC 1.13.11.18) (Ethylmalonic encephalopathy protein 1 homolog) (Hepatoma subtracted clone one protein) (Sulfur dioxygenase ETHE1) (Q9DCM0)
Organism: Mus musculus (Mouse)
Function: First described as a protein that can shuttle between the nucleus and the cytoplasm and suppress p53-induced apoptosis by sequestering the transcription factor RELA/NFKB3 in the cytoplasm and preventing its accumulation in the nucleus (By similarity). Sulfur dioxygenase that plays an essential role in hydrogen sulfide catabolism in the mitochondrial matrix. Hydrogen sulfide (H(2)S) is first oxidized by SQRDL, giving rise to cysteine persulfide residues. ETHE1 consumes molecular oxygen to catalyze the oxidation of the persulfide, once it has been transferred to a thiophilic acceptor, such as glutathione (R-SSH). Plays an important role in metabolic homeostasis in mitochondria by metabolizing hydrogen sulfide and preventing the accumulation of supraphysiological H(2)S levels that have toxic effects, due to the inhibition of cytochrome c oxidase.
Subcellular location: Cytoplasm {ECO:0000250|UniProtKB:O95571}. Nucleus {ECO:0000250|UniProtKB:O95571}. Mitochondrion matrix {ECO:0000250|UniProtKB:O95571}.
Sequence: MASAVVRVAGRRLSQQSASGAPVLLRQMFEPKSCTYTYLLGDRESREAVLIDPVLETAHRDAQLIKELGLKLLYAVNTHCHADHITGTGVLRSLLPGCQSVISRLSGAQADLHIGEGDSIRFGRFALETRASPGHTPGCVTFVLNDQSMAFTGDALLIRGCGRTDFQQGCAKTLYHSVHEKIFTLPGNCLIYPAHDYHGLTVSTVEEERTLNPRLTLSCEEFIKVMDNLNLPKPQQIDIAVPANMRCGVQTPPS
InterPro domains: - IPR001279: Metallo-beta-lactamase (domain) [34-195]
- IPR044528: Persulfide dioxygenase-like, MBL-fold metallo-hydrolase domain (domain) [24-197]
- IPR036866: Ribonuclease Z/Hydroxyacylglutathione hydrolase-like (homologous_superfamily) [18-249]
- IPR051682: Mitochondrial Persulfide Dioxygenase (family) [15-251]
Interaction partners: - Sulfite oxidase, mitochondrial
- Sulfide:quinone oxidoreductase, mitochondrial
- Thiosulfate sulfurtransferase
- 3-mercaptopyruvate sulfurtransferase
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0003824 (catalytic activity), GO:0016491 (oxidoreductase activity), GO:0051213 (dioxygenase activity), GO:0016701 (oxidoreductase activity, acting on single donors with incorporation of molecular oxygen), GO:0016702 (oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0009987 (cellular process), GO:0044237 (cellular metabolic process), GO:0071704 (organic substance metabolic process), GO:0044281 (small molecule metabolic process), GO:0009056 (catabolic process), GO:0042180 (cellular ketone metabolic process), GO:1901615 (organic hydroxy compound metabolic process), GO:0044282 (small molecule catabolic process), GO:1901575 (organic substance catabolic process), GO:0006081 (cellular aldehyde metabolic process), GO:0044248 (cellular catabolic process), GO:0006082 (organic acid metabolic process), GO:0006089 (lactate metabolic process), GO:0042182 (ketone catabolic process), GO:0046185 (aldehyde catabolic process), GO:0009438 (methylglyoxal metabolic process), GO:0043436 (oxoacid metabolic process), GO:0019752 (carboxylic acid metabolic process), GO:0051596 (methylglyoxal catabolic process), GO:0032787 (monocarboxylic acid metabolic process)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0005737 (cytoplasm), GO:0043229 (intracellular organelle), GO:0005739 (mitochondrion), GO:0043227 (membrane-bounded organelle), GO:0043231 (intracellular membrane-bounded organelle)