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Q9TY03

- FRDA_CAEEL

UniProt

Q9TY03 - FRDA_CAEEL

Protein

Frataxin, mitochondrial

Gene

frh-1

Organism
Caenorhabditis elegans
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at transcript leveli
  1. Functioni

    Promotes the biosynthesis of heme as well as the assembly and repair of iron-sulfur clusters by delivering Fe2+ to proteins involved in these pathways. May play a role in the protection against iron-catalyzed oxidative stress through its ability to catalyze the oxidation of Fe2+ to Fe3+. May be able to store large amounts of the metal in the form of a ferrihydrite mineral by oligomerization.1 Publication

    Catalytic activityi

    4 Fe2+ + 4 H+ + O2 = 4 Fe3+ + 2 H2O.

    GO - Molecular functioni

    1. ferric iron binding Source: InterPro
    2. ferroxidase activity Source: UniProtKB-EC

    GO - Biological processi

    1. cellular iron ion homeostasis Source: UniProtKB-KW
    2. determination of adult lifespan Source: WormBase
    3. embryo development Source: WormBase
    4. growth Source: WormBase
    5. heme biosynthetic process Source: UniProtKB-KW
    6. ion transport Source: UniProtKB-KW
    7. iron-sulfur cluster assembly Source: WormBase
    8. nematode larval development Source: WormBase
    9. response to hydrogen peroxide Source: WormBase
    10. response to superoxide Source: WormBase

    Keywords - Molecular functioni

    Oxidoreductase

    Keywords - Biological processi

    Heme biosynthesis, Ion transport, Iron storage, Iron transport, Transport

    Keywords - Ligandi

    Iron

    Enzyme and pathway databases

    ReactomeiREACT_183224. Mitochondrial iron-sulfur cluster biogenesis.
    REACT_183227. Mitochondrial protein import.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Frataxin, mitochondrial (EC:1.16.3.1)
    Short name:
    Fxn
    Gene namesi
    Name:frh-1
    ORF Names:F59G1.7
    OrganismiCaenorhabditis elegans
    Taxonomic identifieri6239 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaEcdysozoaNematodaChromadoreaRhabditidaRhabditoideaRhabditidaePeloderinaeCaenorhabditis
    ProteomesiUP000001940: Chromosome II

    Organism-specific databases

    WormBaseiF59G1.7; CE19476; WBGene00001486; frh-1.

    Subcellular locationi

    Mitochondrion By similarity

    GO - Cellular componenti

    1. mitochondrion Source: WormBase

    Keywords - Cellular componenti

    Mitochondrion

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini? – 136Frataxin, mitochondrialPRO_0000193925
    Transit peptidei1 – ?MitochondrionSequence Analysis

    Proteomic databases

    PaxDbiQ9TY03.

    Interactioni

    Subunit structurei

    Monomer. Oligomer By similarity.By similarity

    Protein-protein interaction databases

    IntActiQ9TY03. 1 interaction.
    STRINGi6239.F59G1.7.2.

    Structurei

    3D structure databases

    ProteinModelPortaliQ9TY03.
    SMRiQ9TY03. Positions 22-133.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Belongs to the frataxin family.Curated

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG1965.
    GeneTreeiENSGT00390000005811.
    HOGENOMiHOG000190729.
    InParanoidiQ9TY03.
    OMAiYEVEYHS.
    OrthoDBiEOG7HHWVD.
    PhylomeDBiQ9TY03.

    Family and domain databases

    Gene3Di3.30.920.10. 1 hit.
    InterProiIPR017789. Frataxin.
    IPR002908. Frataxin/CyaY.
    IPR020895. Frataxin_CS.
    [Graphical view]
    PANTHERiPTHR16821. PTHR16821. 1 hit.
    PfamiPF01491. Frataxin_Cyay. 1 hit.
    [Graphical view]
    PRINTSiPR00904. FRATAXIN.
    SUPFAMiSSF55387. SSF55387. 1 hit.
    TIGRFAMsiTIGR03421. FeS_CyaY. 1 hit.
    TIGR03422. mito_frataxin. 1 hit.
    PROSITEiPS01344. FRATAXIN_1. 1 hit.
    PS50810. FRATAXIN_2. 1 hit.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

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

    Q9TY03-1 [UniParc]FASTAAdd to Basket

    « Hide

    MLSTILRNNF VRRSFSSRIF SQNEYETAAD STLERLSDYF DQIADSFPVS    50
    EQFDVSHAMG VLTVNVSKSV GTYVINKQSP NKQIWLSSPM SGPKRYDLEE 100
    EGKWTYAHDG EQLDSLLNRE FRKILADDRI DFSRHV 136
    Length:136
    Mass (Da):15,718
    Last modified:May 1, 2000 - v1
    Checksum:i0ACB254346E38272
    GO

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AY048153 mRNA. Translation: AAL05950.1.
    FO081244 Genomic DNA. Translation: CCD70156.1.
    PIRiT34316.
    RefSeqiNP_495183.1. NM_062782.3.
    UniGeneiCel.20708.

    Genome annotation databases

    EnsemblMetazoaiF59G1.7; F59G1.7; WBGene00001486.
    GeneIDi174002.
    KEGGicel:CELE_F59G1.7.
    UCSCiF59G1.7.1. c. elegans.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AY048153 mRNA. Translation: AAL05950.1 .
    FO081244 Genomic DNA. Translation: CCD70156.1 .
    PIRi T34316.
    RefSeqi NP_495183.1. NM_062782.3.
    UniGenei Cel.20708.

    3D structure databases

    ProteinModelPortali Q9TY03.
    SMRi Q9TY03. Positions 22-133.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    IntActi Q9TY03. 1 interaction.
    STRINGi 6239.F59G1.7.2.

    Proteomic databases

    PaxDbi Q9TY03.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    EnsemblMetazoai F59G1.7 ; F59G1.7 ; WBGene00001486 .
    GeneIDi 174002.
    KEGGi cel:CELE_F59G1.7.
    UCSCi F59G1.7.1. c. elegans.

    Organism-specific databases

    CTDi 174002.
    WormBasei F59G1.7 ; CE19476 ; WBGene00001486 ; frh-1.

    Phylogenomic databases

    eggNOGi COG1965.
    GeneTreei ENSGT00390000005811.
    HOGENOMi HOG000190729.
    InParanoidi Q9TY03.
    OMAi YEVEYHS.
    OrthoDBi EOG7HHWVD.
    PhylomeDBi Q9TY03.

    Enzyme and pathway databases

    Reactomei REACT_183224. Mitochondrial iron-sulfur cluster biogenesis.
    REACT_183227. Mitochondrial protein import.

    Miscellaneous databases

    NextBioi 882063.
    PROi Q9TY03.

    Family and domain databases

    Gene3Di 3.30.920.10. 1 hit.
    InterProi IPR017789. Frataxin.
    IPR002908. Frataxin/CyaY.
    IPR020895. Frataxin_CS.
    [Graphical view ]
    PANTHERi PTHR16821. PTHR16821. 1 hit.
    Pfami PF01491. Frataxin_Cyay. 1 hit.
    [Graphical view ]
    PRINTSi PR00904. FRATAXIN.
    SUPFAMi SSF55387. SSF55387. 1 hit.
    TIGRFAMsi TIGR03421. FeS_CyaY. 1 hit.
    TIGR03422. mito_frataxin. 1 hit.
    PROSITEi PS01344. FRATAXIN_1. 1 hit.
    PS50810. FRATAXIN_2. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "Characterization of the Caenorhabditis elegans ortholog of the frataxin."
      Vazquez-Manrique R.P., Baylis H.A., Palau F.
      Submitted (JUL-2001) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [MRNA].
      Strain: Bristol N2.
    2. "Genome sequence of the nematode C. elegans: a platform for investigating biology."
      The C. elegans sequencing consortium
      Science 282:2012-2018(1998) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
      Strain: Bristol N2.
    3. "Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant."
      Vazquez-Manrique R.P., Gonzalez-Cabo P., Ros S., Aziz H., Baylis H.A., Palau F.
      FASEB J. 20:172-174(2006) [PubMed] [Europe PMC] [Abstract]
      Cited for: FUNCTION.

    Entry informationi

    Entry nameiFRDA_CAEEL
    AccessioniPrimary (citable) accession number: Q9TY03
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: December 1, 2000
    Last sequence update: May 1, 2000
    Last modified: October 1, 2014
    This is version 85 of the entry and version 1 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programCaenorhabditis annotation project

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Reference proteome

    Documents

    1. Caenorhabditis elegans
      Caenorhabditis elegans: entries, gene names and cross-references to WormBase
    2. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3