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Q8HXX9

- FRDA_MACFA

UniProt

Q8HXX9 - FRDA_MACFA

Protein

Frataxin, mitochondrial

Gene

FXN

Organism
Macaca fascicularis (Crab-eating macaque) (Cynomolgus monkey)
Status
Reviewed - Annotation score: 3 out of 5- Experimental evidence at transcript leveli
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    • History
      Entry version 54 (01 Oct 2014)
      Sequence version 1 (01 Mar 2003)
      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. ferric iron binding Source: InterPro
    2. ferroxidase activity Source: UniProtKB-EC

    GO - Biological processi

    1. cellular iron ion homeostasis Source: UniProtKB-KW
    2. heme biosynthetic process Source: UniProtKB-KW
    3. ion transport Source: UniProtKB-KW
    4. iron-sulfur cluster assembly Source: InterPro

    Keywords - Molecular functioni

    Oxidoreductase

    Keywords - Biological processi

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

    Keywords - Ligandi

    Iron

    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:FRDA1
    ORF Names:QnpA-13971
    OrganismiMacaca fascicularis (Crab-eating macaque) (Cynomolgus monkey)
    Taxonomic identifieri9541 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniCercopithecidaeCercopithecinaeMacaca

    Subcellular locationi

    Cytoplasm By similarity. Mitochondrion By similarity

    GO - Cellular componenti

    1. mitochondrion Source: UniProtKB-SubCell

    Keywords - Cellular componenti

    Cytoplasm, Mitochondrion

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 4141MitochondrionBy similarityAdd
    BLAST
    Chaini42 – 210169Frataxin intermediate formPRO_0000010131Add
    BLAST
    Chaini81 – 210130Frataxin mature formBy similarityPRO_0000399389Add
    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

    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

    Structurei

    3D structure databases

    ProteinModelPortaliQ8HXX9.
    SMRiQ8HXX9. Positions 90-210.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Sequence similaritiesi

    Belongs to the frataxin family.Curated

    Keywords - Domaini

    Transit peptide

    Phylogenomic databases

    HOVERGENiHBG005745.

    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.

    Q8HXX9-1 [UniParc]FASTAAdd to Basket

    « Hide

    MWTFGRRAVA GLLASPSPAQ AQTLTRAPRL AELAQLCSRR GLRTGINATR    50
    TTHHTSSNLR GLNQIRNVKR QSVYLMNLRK SGTLGHPGSL DDTTYERLAE 100
    ETLDSLAEFF EDLADKPYTF EDYDVSFGSG VLTVKLGGDL GTYVINKQTP 150
    NKQIWLSSPS SGPKRYDRTG KNWVYSHDGV SLHELLGAEL TKALKTKLDL 200
    SSLAYSGKDA 210
    Length:210
    Mass (Da):23,133
    Last modified:March 1, 2003 - v1
    Checksum:iC90A50A3F3E7D581
    GO

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AB083310 mRNA. Translation: BAC20589.1.
    RefSeqiNP_001271967.1. NM_001285038.1.
    UniGeneiMfa.6270.

    Genome annotation databases

    GeneIDi102120656.
    KEGGimcf:102120656.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AB083310 mRNA. Translation: BAC20589.1 .
    RefSeqi NP_001271967.1. NM_001285038.1.
    UniGenei Mfa.6270.

    3D structure databases

    ProteinModelPortali Q8HXX9.
    SMRi Q8HXX9. Positions 90-210.
    ModBasei Search...
    MobiDBi Search...

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    GeneIDi 102120656.
    KEGGi mcf:102120656.

    Organism-specific databases

    CTDi 2395.

    Phylogenomic databases

    HOVERGENi HBG005745.

    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. "Isolation and characterization of cDNA for macaque neurological disease genes."
      Kusuda J., Osada N., Hida M., Sugano S., Hashimoto K.
      Submitted (APR-2002) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Tissue: Parietal cortex.

    Entry informationi

    Entry nameiFRDA_MACFA
    AccessioniPrimary (citable) accession number: Q8HXX9
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: April 12, 2005
    Last sequence update: March 1, 2003
    Last modified: October 1, 2014
    This is version 54 of the entry and version 1 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    Miscellaneousi

    Documents

    1. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3