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Q9DCM0

- ETHE1_MOUSE

UniProt

Q9DCM0 - ETHE1_MOUSE

Protein

Persulfide dioxygenase ETHE1, mitochondrial

Gene

Ethe1

Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 92 (01 Oct 2014)
      Sequence version 2 (01 Mar 2003)
      Previous versions | rss
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    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

    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. hydrolase activity Source: InterPro
    2. iron ion binding Source: UniProtKB
    3. sulfur dioxygenase activity Source: UniProtKB

    GO - Biological processi

    1. glutathione metabolic process Source: UniProtKB
    2. hydrogen sulfide metabolic process Source: UniProtKB

    Keywords - Molecular functioni

    Dioxygenase, Oxidoreductase

    Keywords - Ligandi

    Metal-binding

    Enzyme and pathway databases

    ReactomeiREACT_198963. 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: Chromosome 7

    Organism-specific databases

    MGIiMGI:1913321. Ethe1.

    Subcellular locationi

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

    GO - Cellular componenti

    1. mitochondrial matrix Source: UniProtKB-SubCell
    2. mitochondrion Source: MGI
    3. nucleus Source: UniProtKB-SubCell

    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 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

    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.
    OMAiNPKMMDV.
    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

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    MASAVVRVAG RRLSQQSASG APVLLRQMFE PKSCTYTYLL GDRESREAVL    50
    IDPVLETAHR DAQLIKELGL KLLYAVNTHC HADHITGTGV LRSLLPGCQS 100
    VISRLSGAQA DLHIGEGDSI RFGRFALETR ASPGHTPGCV TFVLNDQSMA 150
    FTGDALLIRG CGRTDFQQGC AKTLYHSVHE KIFTLPGNCL IYPAHDYHGL 200
    TVSTVEEERT LNPRLTLSCE EFIKVMDNLN LPKPQQIDIA VPANMRCGVQ 250
    TPPS 254
    Length:254
    Mass (Da):27,739
    Last modified:March 1, 2003 - v2
    Checksum:i3F28C0ED465A3062
    GO

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    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.
    XP_006540342.1. XM_006540279.1.
    UniGeneiMm.29553.

    Genome annotation databases

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

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    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 .
    CCDSi CCDS20957.1.
    RefSeqi NP_075643.1. NM_023154.3.
    XP_006540342.1. XM_006540279.1.
    UniGenei Mm.29553.

    3D structure databases

    ProteinModelPortali Q9DCM0.
    SMRi Q9DCM0. Positions 23-245.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    IntActi Q9DCM0. 2 interactions.
    MINTi MINT-1850653.
    STRINGi 10090.ENSMUSP00000076433.

    PTM databases

    PhosphoSitei Q9DCM0.

    2D gel databases

    REPRODUCTION-2DPAGE Q9DCM0.

    Proteomic databases

    MaxQBi Q9DCM0.
    PaxDbi Q9DCM0.
    PRIDEi Q9DCM0.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENSMUST00000077191 ; ENSMUSP00000076433 ; ENSMUSG00000064254 .
    GeneIDi 66071.
    KEGGi mmu:66071.
    UCSCi uc009fqb.2. mouse.

    Organism-specific databases

    CTDi 23474.
    MGIi MGI:1913321. Ethe1.

    Phylogenomic databases

    eggNOGi COG0491.
    GeneTreei ENSGT00530000063033.
    HOGENOMi HOG000058040.
    HOVERGENi HBG053310.
    InParanoidi Q9DCM0.
    KOi K17725.
    OMAi NPKMMDV.
    OrthoDBi EOG7MH107.
    PhylomeDBi Q9DCM0.
    TreeFami TF312952.

    Enzyme and pathway databases

    Reactomei REACT_198963. Sulfide oxidation to sulfate.

    Miscellaneous databases

    ChiTaRSi ETHE1. mouse.
    NextBioi 320540.
    PROi Q9DCM0.
    SOURCEi Search...

    Gene expression databases

    Bgeei Q9DCM0.
    CleanExi MM_ETHE1.
    Genevestigatori Q9DCM0.

    Family and domain databases

    Gene3Di 3.60.15.10. 1 hit.
    InterProi IPR001279. Beta-lactamas-like.
    [Graphical view ]
    Pfami PF00753. Lactamase_B. 1 hit.
    [Graphical view ]
    SMARTi SM00849. Lactamase_B. 1 hit.
    [Graphical view ]
    SUPFAMi SSF56281. SSF56281. 1 hit.
    ProtoNeti Search...

    Publicationsi

    1. "Expression of HSCO in hepatomas."
      Fujita J., Higashitsuji H., Higashitsuji H.
      Submitted (OCT-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(2005) [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(2004) [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(2009) [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(2013) [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(2013) [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: October 1, 2014
    This is version 92 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