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Protein

Persulfide dioxygenase ETHE1, mitochondrial

Gene

Ethe1

Organism
Mus musculus (Mouse)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

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 (H2S) 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 H2S levels that have toxic effects, due to the inhibition of cytochrome c oxidase.By similarity1 Publication

Catalytic activityi

Sulfur + O2 + H2O = sulfite + 2 H+.

Cofactori

Fe2+By similarityNote: Binds 1 Fe2+ ion per subunit.By similarity

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Metal bindingi79 – 791Iron; catalyticBy similarity
Metal bindingi135 – 1351Iron; catalyticBy similarity
Metal bindingi154 – 1541Iron; catalyticBy similarity

GO - Molecular functioni

  1. iron ion binding Source: UniProtKB
  2. sulfur dioxygenase activity Source: UniProtKB

GO - Biological processi

  1. glutathione metabolic process Source: UniProtKB
  2. hydrogen sulfide metabolic process Source: UniProtKB
Complete GO annotation...

Keywords - Molecular functioni

Dioxygenase, Oxidoreductase

Keywords - Ligandi

Metal-binding

Enzyme and pathway databases

ReactomeiREACT_273024. Sulfide oxidation to sulfate.

Names & Taxonomyi

Protein namesi
Recommended name:
Persulfide dioxygenase ETHE1, mitochondrial (EC:1.13.11.18)
Alternative name(s):
Ethylmalonic encephalopathy protein 1 homolog
Hepatoma subtracted clone one protein
Sulfur dioxygenase ETHE1
Gene namesi
Name:Ethe1
Synonyms:Hsco
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589 Componenti: Chromosome 7

Organism-specific databases

MGIiMGI:1913321. Ethe1.

Subcellular locationi

Cytoplasm By similarity. Nucleus By similarity. Mitochondrion matrix By similarity

GO - Cellular componenti

  1. cytoplasm Source: MGI
  2. mitochondrial matrix Source: UniProtKB-SubCell
  3. mitochondrion Source: MGI
  4. nucleoplasm Source: MGI
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Mitochondrion, Nucleus

Pathology & Biotechi

Disruption phenotypei

Mice are born at slightly less than the expected Mendelian rate. Pups display growth arrest at about 15 days after birth and die five to six weeks after birth. Mice exhibit elevated levels of hydrogen sulfide (H2S) in liver, muscle and brain, together with increased urinary levels of ethylmalonic acid and thiosulfate. Their mitochondria show decreased cytochrome c oxidase activity, probably due to the toxic effects of supraphysiological levels of hydrogen sulfide.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Transit peptidei1 – 77MitochondrionBy similarity
Chaini8 – 254247Persulfide dioxygenase ETHE1, mitochondrialPRO_0000012290Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei19 – 191PhosphoserineBy similarity
Modified residuei32 – 321N6-acetyllysine; alternate1 Publication
Modified residuei32 – 321N6-succinyllysine; alternate1 Publication
Modified residuei66 – 661N6-acetyllysine1 Publication
Modified residuei172 – 1721N6-acetyllysine; alternate1 Publication
Modified residuei172 – 1721N6-succinyllysine; alternate1 Publication

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

MaxQBiQ9DCM0.
PaxDbiQ9DCM0.
PRIDEiQ9DCM0.

2D gel databases

REPRODUCTION-2DPAGEQ9DCM0.

PTM databases

PhosphoSiteiQ9DCM0.

Expressioni

Gene expression databases

BgeeiQ9DCM0.
CleanExiMM_ETHE1.
GenevestigatoriQ9DCM0.

Interactioni

Subunit structurei

Monomer. Interacts with TST. May interact with RELA (By similarity).By similarity

Protein-protein interaction databases

IntActiQ9DCM0. 2 interactions.
MINTiMINT-1850653.
STRINGi10090.ENSMUSP00000076433.

Structurei

3D structure databases

ProteinModelPortaliQ9DCM0.
SMRiQ9DCM0. Positions 23-245.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG0491.
GeneTreeiENSGT00530000063033.
HOGENOMiHOG000058040.
HOVERGENiHBG053310.
InParanoidiQ9DCM0.
KOiK17725.
OMAiFPAHDYK.
OrthoDBiEOG7MH107.
PhylomeDBiQ9DCM0.
TreeFamiTF312952.

Family and domain databases

Gene3Di3.60.15.10. 1 hit.
InterProiIPR001279. Beta-lactamas-like.
[Graphical view]
PfamiPF00753. Lactamase_B. 1 hit.
[Graphical view]
SMARTiSM00849. Lactamase_B. 1 hit.
[Graphical view]
SUPFAMiSSF56281. SSF56281. 1 hit.

Sequencei

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

Q9DCM0-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MASAVVRVAG RRLSQQSASG APVLLRQMFE PKSCTYTYLL GDRESREAVL
60 70 80 90 100
IDPVLETAHR DAQLIKELGL KLLYAVNTHC HADHITGTGV LRSLLPGCQS
110 120 130 140 150
VISRLSGAQA DLHIGEGDSI RFGRFALETR ASPGHTPGCV TFVLNDQSMA
160 170 180 190 200
FTGDALLIRG CGRTDFQQGC AKTLYHSVHE KIFTLPGNCL IYPAHDYHGL
210 220 230 240 250
TVSTVEEERT LNPRLTLSCE EFIKVMDNLN LPKPQQIDIA VPANMRCGVQ

TPPS
Length:254
Mass (Da):27,739
Last modified:March 1, 2003 - v2
Checksum:i3F28C0ED465A3062
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB049623 mRNA. Translation: BAB16409.1.
AK002666 mRNA. Translation: BAB22271.2.
BC010592 mRNA. Translation: AAH10592.1.
BC083162 mRNA. Translation: AAH83162.1.
BC094044 mRNA. Translation: AAH94044.1.
CCDSiCCDS20957.1.
RefSeqiNP_075643.1. NM_023154.3.
UniGeneiMm.29553.

Genome annotation databases

EnsembliENSMUST00000077191; ENSMUSP00000076433; ENSMUSG00000064254.
GeneIDi66071.
KEGGimmu:66071.
UCSCiuc009fqb.2. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AB049623 mRNA. Translation: BAB16409.1.
AK002666 mRNA. Translation: BAB22271.2.
BC010592 mRNA. Translation: AAH10592.1.
BC083162 mRNA. Translation: AAH83162.1.
BC094044 mRNA. Translation: AAH94044.1.
CCDSiCCDS20957.1.
RefSeqiNP_075643.1. NM_023154.3.
UniGeneiMm.29553.

3D structure databases

ProteinModelPortaliQ9DCM0.
SMRiQ9DCM0. Positions 23-245.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiQ9DCM0. 2 interactions.
MINTiMINT-1850653.
STRINGi10090.ENSMUSP00000076433.

PTM databases

PhosphoSiteiQ9DCM0.

2D gel databases

REPRODUCTION-2DPAGEQ9DCM0.

Proteomic databases

MaxQBiQ9DCM0.
PaxDbiQ9DCM0.
PRIDEiQ9DCM0.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000077191; ENSMUSP00000076433; ENSMUSG00000064254.
GeneIDi66071.
KEGGimmu:66071.
UCSCiuc009fqb.2. mouse.

Organism-specific databases

CTDi23474.
MGIiMGI:1913321. Ethe1.

Phylogenomic databases

eggNOGiCOG0491.
GeneTreeiENSGT00530000063033.
HOGENOMiHOG000058040.
HOVERGENiHBG053310.
InParanoidiQ9DCM0.
KOiK17725.
OMAiFPAHDYK.
OrthoDBiEOG7MH107.
PhylomeDBiQ9DCM0.
TreeFamiTF312952.

Enzyme and pathway databases

ReactomeiREACT_273024. Sulfide oxidation to sulfate.

Miscellaneous databases

ChiTaRSiEthe1. mouse.
NextBioi320540.
PROiQ9DCM0.
SOURCEiSearch...

Gene expression databases

BgeeiQ9DCM0.
CleanExiMM_ETHE1.
GenevestigatoriQ9DCM0.

Family and domain databases

Gene3Di3.60.15.10. 1 hit.
InterProiIPR001279. Beta-lactamas-like.
[Graphical view]
PfamiPF00753. Lactamase_B. 1 hit.
[Graphical view]
SMARTiSM00849. Lactamase_B. 1 hit.
[Graphical view]
SUPFAMiSSF56281. SSF56281. 1 hit.
ProtoNetiSearch...

Publicationsi

« Hide 'large scale' publications
  1. "Expression of HSCO in hepatomas."
    Fujita J., Higashitsuji H., Higashitsuji H.
    Submitted (SEP-2000) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Tissue: Liver.
  2. "The transcriptional landscape of the mammalian genome."
    Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.
    , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.
    Science 309:1559-1563(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Strain: C57BL/6J.
    Tissue: Kidney.
  3. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2003) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Strain: FVB/N.
    Tissue: Colon, Kidney and Mammary tumor.
  4. "Loss of ETHE1, a mitochondrial dioxygenase, causes fatal sulfide toxicity in ethylmalonic encephalopathy."
    Tiranti V., Viscomi C., Hildebrandt T., Di Meo I., Mineri R., Tiveron C., Levitt M.D., Prelle A., Fagiolari G., Rimoldi M., Zeviani M.
    Nat. Med. 15:200-205(2008) [PubMed] [Europe PMC] [Abstract]
    Cited for: DISRUPTION PHENOTYPE, FUNCTION.
  5. "SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways."
    Park J., Chen Y., Tishkoff D.X., Peng C., Tan M., Dai L., Xie Z., Zhang Y., Zwaans B.M., Skinner M.E., Lombard D.B., Zhao Y.
    Mol. Cell 50:919-930(2012) [PubMed] [Europe PMC] [Abstract]
    Cited for: SUCCINYLATION [LARGE SCALE ANALYSIS] AT LYS-32 AND LYS-172, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.
  6. "Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways."
    Rardin M.J., Newman J.C., Held J.M., Cusack M.P., Sorensen D.J., Li B., Schilling B., Mooney S.D., Kahn C.R., Verdin E., Gibson B.W.
    Proc. Natl. Acad. Sci. U.S.A. 110:6601-6606(2012) [PubMed] [Europe PMC] [Abstract]
    Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-32; LYS-66 AND LYS-172, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Liver.

Entry informationi

Entry nameiETHE1_MOUSE
AccessioniPrimary (citable) accession number: Q9DCM0
Secondary accession number(s): Q9ESL5
Entry historyi
Integrated into UniProtKB/Swiss-Prot: August 30, 2005
Last sequence update: March 1, 2003
Last modified: April 1, 2015
This is version 98 of the entry and version 2 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into Uniref entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.