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Protein

Frataxin, mitochondrial

Gene

Fxn

Organism
Rattus norvegicus (Rat)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein 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 similarity1 Publication

Catalytic activityi

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

GO - Molecular functioni

  • enzyme binding Source: RGD
  • ferric iron binding Source: InterPro
  • ferroxidase activity Source: UniProtKB-EC
  • metallochaperone activity Source: RGD

GO - Biological processi

  • cellular iron ion homeostasis Source: RGD
  • heme biosynthetic process Source: UniProtKB-KW
  • ion transport Source: UniProtKB-KW
  • iron-sulfur cluster assembly Source: InterPro
  • positive regulation of axon extension Source: RGD
  • response to drug Source: RGD
  • response to organic cyclic compound Source: RGD
Complete GO annotation...

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
OrganismiRattus norvegicus (Rat)
Taxonomic identifieri10116 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeRattus
Proteomesi
  • UP000002494 Componenti: Unplaced

Organism-specific databases

RGDi1565754. Fxn.

Subcellular locationi

  • Cytoplasmcytosol By similarity
  • Mitochondrion By similarity

GO - Cellular componenti

  • cytosol Source: UniProtKB-SubCell
  • mitochondrion Source: RGD
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 40MitochondrionBy similarityAdd BLAST40
ChainiPRO_000039946541 – 208Frataxin intermediate formAdd BLAST168
ChainiPRO_000039939179 – 208Frataxin mature formBy similarityAdd BLAST130

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

PeptideAtlasiD3ZYW7.
PRIDEiD3ZYW7.

PTM databases

iPTMnetiD3ZYW7.
PhosphoSitePlusiD3ZYW7.

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 (By similarity). Interacts with ACO2; the interaction is dependent on citrate.By similarity1 Publication

GO - Molecular functioni

  • enzyme binding Source: RGD

Family & Domainsi

Sequence similaritiesi

Belongs to the frataxin family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

InParanoidiD3ZYW7.
PhylomeDBiD3ZYW7.
TreeFamiTF318958.

Family and domain databases

Gene3Di3.30.920.10. 1 hit.
InterProiIPR017789. Frataxin.
IPR002908. Frataxin/CyaY.
[Graphical view]
PANTHERiPTHR16821. PTHR16821. 1 hit.
PfamiPF01491. Frataxin_Cyay. 1 hit.
[Graphical view]
PRINTSiPR00904. FRATAXIN.
SMARTiSM01219. Frataxin_Cyay. 1 hit.
[Graphical view]
SUPFAMiSSF55387. SSF55387. 1 hit.
TIGRFAMsiTIGR03421. FeS_CyaY. 1 hit.
TIGR03422. mito_frataxin. 1 hit.
PROSITEiPS50810. FRATAXIN_2. 1 hit.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

D3ZYW7-1 [UniParc]FASTAAdd to basket

« Hide

        10         20         30         40         50
MWTFGRRAAA GLLPRTASRA SAWVRNPRGR ERIGTCGRRG LHVTANADAI
60 70 80 90 100
RHSHLNLHYL GQILNIKKQS VCVVHLRNSG TLGNPSSLDE TAYERLAEET
110 120 130 140 150
LDALAEFFED LADKPYTLKD YDVSFGDGVL TIKLGGDLGT YVINKQTPLL
160 170 180 190 200
YLWFSGPCSG PKRYDWTGKN WVYSHDGVSL HELLARELTE ALNTKLDLSS

LAYSGKGT
Length:208
Mass (Da):23,066
Last modified:April 20, 2010 - v1
Checksum:i401B8210F5CEDD35
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AABR03005338 Genomic DNA. No translation available.
AABR03006217 Genomic DNA. No translation available.
AABR03009513 Genomic DNA. No translation available.
UniGeneiRn.13133.

Genome annotation databases

UCSCiRGD:1565754. rat.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AABR03005338 Genomic DNA. No translation available.
AABR03006217 Genomic DNA. No translation available.
AABR03009513 Genomic DNA. No translation available.
UniGeneiRn.13133.

3D structure databases

ModBaseiSearch...
MobiDBiSearch...

PTM databases

iPTMnetiD3ZYW7.
PhosphoSitePlusiD3ZYW7.

Proteomic databases

PeptideAtlasiD3ZYW7.
PRIDEiD3ZYW7.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

UCSCiRGD:1565754. rat.

Organism-specific databases

RGDi1565754. Fxn.

Phylogenomic databases

InParanoidiD3ZYW7.
PhylomeDBiD3ZYW7.
TreeFamiTF318958.

Miscellaneous databases

PROiD3ZYW7.

Family and domain databases

Gene3Di3.30.920.10. 1 hit.
InterProiIPR017789. Frataxin.
IPR002908. Frataxin/CyaY.
[Graphical view]
PANTHERiPTHR16821. PTHR16821. 1 hit.
PfamiPF01491. Frataxin_Cyay. 1 hit.
[Graphical view]
PRINTSiPR00904. FRATAXIN.
SMARTiSM01219. Frataxin_Cyay. 1 hit.
[Graphical view]
SUPFAMiSSF55387. SSF55387. 1 hit.
TIGRFAMsiTIGR03421. FeS_CyaY. 1 hit.
TIGR03422. mito_frataxin. 1 hit.
PROSITEiPS50810. FRATAXIN_2. 1 hit.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiFRDA_RAT
AccessioniPrimary (citable) accession number: D3ZYW7
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 5, 2010
Last sequence update: April 20, 2010
Last modified: November 2, 2016
This is version 40 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

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 one 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.