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Q05B87

- FRDA_BOVIN

UniProt

Q05B87 - FRDA_BOVIN

Protein

Frataxin, mitochondrial

Gene

FXN

Organism
Bos taurus (Bovine)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at transcript leveli
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    • History
      Entry version 52 (01 Oct 2014)
      Sequence version 1 (14 Nov 2006)
      Previous versions | rss
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    Functioni

    Promotes the biosynthesis of heme and 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+; the oligomeric form but not the monomeric form has in vitro ferroxidase activity. May be able to store large amounts of iron in the form of a ferrihydrite mineral by oligomerization. Modulates the RNA-binding activity of ACO1 By similarity.By similarity

    Catalytic activityi

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

    GO - Molecular functioni

    1. 2 iron, 2 sulfur cluster binding Source: Ensembl
    2. ferric iron binding Source: InterPro
    3. ferrous iron binding Source: Ensembl
    4. ferroxidase activity Source: UniProtKB-EC
    5. iron chaperone activity Source: Ensembl

    GO - Biological processi

    1. adult walking behavior Source: Ensembl
    2. aerobic respiration Source: Ensembl
    3. cellular iron ion homeostasis Source: UniProtKB-KW
    4. cellular response to hydrogen peroxide Source: Ensembl
    5. embryo development ending in birth or egg hatching Source: Ensembl
    6. heme biosynthetic process Source: UniProtKB-KW
    7. ion transport Source: UniProtKB-KW
    8. iron incorporation into metallo-sulfur cluster Source: Ensembl
    9. mitochondrion organization Source: Ensembl
    10. negative regulation of apoptotic process Source: Ensembl
    11. negative regulation of multicellular organism growth Source: Ensembl
    12. negative regulation of organ growth Source: Ensembl
    13. negative regulation of release of cytochrome c from mitochondria Source: Ensembl
    14. oxidative phosphorylation Source: Ensembl
    15. positive regulation of cell growth Source: Ensembl
    16. positive regulation of cell proliferation Source: Ensembl
    17. positive regulation of lyase activity Source: Ensembl
    18. positive regulation of metalloenzyme activity Source: Ensembl
    19. positive regulation of oxidoreductase activity Source: Ensembl
    20. positive regulation of transferase activity Source: Ensembl
    21. proprioception Source: Ensembl
    22. protein autoprocessing Source: Ensembl
    23. regulation of ferrochelatase activity Source: Ensembl
    24. response to iron ion Source: Ensembl

    Keywords - Molecular functioni

    Oxidoreductase

    Keywords - Biological processi

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

    Keywords - Ligandi

    Iron

    Enzyme and pathway databases

    ReactomeiREACT_215731. Mitochondrial protein import.
    REACT_215986. Mitochondrial iron-sulfur cluster biogenesis.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Frataxin, mitochondrial (EC:1.16.3.1)
    Short name:
    Fxn
    Cleaved into the following 2 chains:
    Gene namesi
    Name:FXN
    Synonyms:FRDA
    OrganismiBos taurus (Bovine)
    Taxonomic identifieri9913 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaLaurasiatheriaCetartiodactylaRuminantiaPecoraBovidaeBovinaeBos
    ProteomesiUP000009136: Chromosome 8

    Subcellular locationi

    Cytoplasm By similarity. Mitochondrion By similarity

    GO - Cellular componenti

    1. cytosol Source: Ensembl
    2. mitochondrion Source: UniProtKB-SubCell

    Keywords - Cellular componenti

    Cytoplasm, Mitochondrion

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 4242MitochondrionBy similarityAdd
    BLAST
    Chaini43 – 217175Frataxin intermediate formPRO_0000289330Add
    BLAST
    Chaini82 – 217136Frataxin mature formBy similarityPRO_0000399387Add
    BLAST

    Post-translational modificationi

    Processed in two steps by mitochondrial processing peptidase (MPP). MPP first cleaves the precursor to intermediate form and subsequently converts the intermediate to yield frataxin mature form By similarity.By similarity

    Proteomic databases

    PRIDEiQ05B87.

    Interactioni

    Subunit structurei

    Monomer (probable predominant form). Oligomer. Interacts with LYRM4 AND HSPA9. Interacts with ACO1. Interacts with ISCU. Interacts with FECH; one iron-bound FXN monomer seems to interact with a FECH homodimer. Interacts with SDHA and SDHB. Interacts with ACO2; the interaction is dependent on citrate By similarity.By similarity

    Protein-protein interaction databases

    STRINGi9913.ENSBTAP00000001725.

    Structurei

    3D structure databases

    ProteinModelPortaliQ05B87.
    SMRiQ05B87. Positions 91-210.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Belongs to the frataxin family.Curated

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    eggNOGiCOG1965.
    GeneTreeiENSGT00390000005811.
    HOGENOMiHOG000190729.
    HOVERGENiHBG005745.
    InParanoidiQ05B87.
    OMAiKQSVCLM.
    OrthoDBiEOG7HHWVD.
    TreeFamiTF318958.

    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.

    Q05B87-1 [UniParc]FASTAAdd to Basket

    « Hide

    MWTLGRRSVA SFLPRSALPG FAPTRAGAPR PAKDLSLSGL PGLRIGTAKA    50
    PARSQSSLSL RCLNQTLDVK KQSVCWINLR TAGTLGDAGT LDDTTYERLA 100
    EETLDSLAEF FEDLADKPYT FEDYDVSFGS GVLTVKLGGD LGTYVINKQT 150
    PNKQIWLSSP SSGPKRYDWT GRNWVYSHDG VSLHELLATE LTQALKTKLD 200
    LSALAYSGKD TCCPAQC 217
    Length:217
    Mass (Da):23,567
    Last modified:November 14, 2006 - v1
    Checksum:iAF8AB00650CD9ACC
    GO

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    BC122620 mRNA. Translation: AAI22621.1.
    RefSeqiNP_001074196.1. NM_001080727.1.
    UniGeneiBt.17029.

    Genome annotation databases

    EnsembliENSBTAT00000001725; ENSBTAP00000001725; ENSBTAG00000001306.
    GeneIDi505694.
    KEGGibta:505694.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    BC122620 mRNA. Translation: AAI22621.1 .
    RefSeqi NP_001074196.1. NM_001080727.1.
    UniGenei Bt.17029.

    3D structure databases

    ProteinModelPortali Q05B87.
    SMRi Q05B87. Positions 91-210.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    STRINGi 9913.ENSBTAP00000001725.

    Proteomic databases

    PRIDEi Q05B87.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENSBTAT00000001725 ; ENSBTAP00000001725 ; ENSBTAG00000001306 .
    GeneIDi 505694.
    KEGGi bta:505694.

    Organism-specific databases

    CTDi 2395.

    Phylogenomic databases

    eggNOGi COG1965.
    GeneTreei ENSGT00390000005811.
    HOGENOMi HOG000190729.
    HOVERGENi HBG005745.
    InParanoidi Q05B87.
    OMAi KQSVCLM.
    OrthoDBi EOG7HHWVD.
    TreeFami TF318958.

    Enzyme and pathway databases

    Reactomei REACT_215731. Mitochondrial protein import.
    REACT_215986. Mitochondrial iron-sulfur cluster biogenesis.

    Miscellaneous databases

    NextBioi 20867270.

    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. NIH - Mammalian Gene Collection (MGC) project
      Submitted (AUG-2006) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Strain: Hereford.
      Tissue: Thalamus.

    Entry informationi

    Entry nameiFRDA_BOVIN
    AccessioniPrimary (citable) accession number: Q05B87
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: May 29, 2007
    Last sequence update: November 14, 2006
    Last modified: October 1, 2014
    This is version 52 of the entry and version 1 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. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3