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Protein

Frataxin, mitochondrial

Gene

FXN

Organism
Bos taurus (Bovine)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at transcript leveli

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 multicellular organism growth Source: Ensembl
  11. negative regulation of organ growth Source: Ensembl
  12. negative regulation of release of cytochrome c from mitochondria Source: Ensembl
  13. oxidative phosphorylation Source: Ensembl
  14. positive regulation of cell growth Source: Ensembl
  15. positive regulation of cell proliferation Source: Ensembl
  16. positive regulation of lyase activity Source: Ensembl
  17. positive regulation of metalloenzyme activity Source: Ensembl
  18. positive regulation of oxidoreductase activity Source: Ensembl
  19. positive regulation of transferase activity Source: Ensembl
  20. proprioception Source: Ensembl
  21. protein autoprocessing Source: Ensembl
  22. regulation of ferrochelatase activity Source: Ensembl
  23. response to iron ion Source: Ensembl
Complete GO annotation...

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
Complete GO annotation...

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

        10         20         30         40         50
MWTLGRRSVA SFLPRSALPG FAPTRAGAPR PAKDLSLSGL PGLRIGTAKA
60 70 80 90 100
PARSQSSLSL RCLNQTLDVK KQSVCWINLR TAGTLGDAGT LDDTTYERLA
110 120 130 140 150
EETLDSLAEF FEDLADKPYT FEDYDVSFGS GVLTVKLGGD LGTYVINKQT
160 170 180 190 200
PNKQIWLSSP SSGPKRYDWT GRNWVYSHDG VSLHELLATE LTQALKTKLD
210
LSALAYSGKD TCCPAQC
Length:217
Mass (Da):23,567
Last modified:November 14, 2006 - v1
Checksum:iAF8AB00650CD9ACC
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
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:
EMBLi
GenBanki
DDBJi
Links Updated
BC122620 mRNA. Translation: AAI22621.1.
RefSeqiNP_001074196.1. NM_001080727.1.
UniGeneiBt.17029.

3D structure databases

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

Protein-protein interaction databases

STRINGi9913.ENSBTAP00000001725.

Proteomic databases

PRIDEiQ05B87.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi2395.

Phylogenomic databases

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

Enzyme and pathway databases

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

Miscellaneous databases

NextBioi20867270.

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]
ProtoNetiSearch...

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: January 7, 2015
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

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. 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.