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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

GO - Biological processi

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

ReactomeiR-BTA-1362409. 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
Proteomesi
  • UP000009136 Componenti: Chromosome 8

Subcellular locationi

GO - Cellular componenti

Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 42MitochondrionBy similarityAdd BLAST42
ChainiPRO_000028933043 – 217Frataxin intermediate formAdd BLAST175
ChainiPRO_000039938782 – 217Frataxin mature formBy similarityAdd BLAST136

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

PaxDbiQ05B87.
PRIDEiQ05B87.

Expressioni

Gene expression databases

BgeeiENSBTAG00000001306.

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.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the frataxin family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiKOG3413. Eukaryota.
COG1965. LUCA.
GeneTreeiENSGT00390000005811.
HOGENOMiHOG000190729.
HOVERGENiHBG005745.
InParanoidiQ05B87.
KOiK19054.
OMAiQSVYLMN.
OrthoDBiEOG091G0UR5.
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.
SMARTiSM01219. Frataxin_Cyay. 1 hit.
[Graphical view]
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.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

STRINGi9913.ENSBTAP00000001725.

Proteomic databases

PaxDbiQ05B87.
PRIDEiQ05B87.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

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

Organism-specific databases

CTDi2395.

Phylogenomic databases

eggNOGiKOG3413. Eukaryota.
COG1965. LUCA.
GeneTreeiENSGT00390000005811.
HOGENOMiHOG000190729.
HOVERGENiHBG005745.
InParanoidiQ05B87.
KOiK19054.
OMAiQSVYLMN.
OrthoDBiEOG091G0UR5.
TreeFamiTF318958.

Enzyme and pathway databases

ReactomeiR-BTA-1362409. Mitochondrial iron-sulfur cluster biogenesis.

Gene expression databases

BgeeiENSBTAG00000001306.

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.
SMARTiSM01219. Frataxin_Cyay. 1 hit.
[Graphical view]
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...

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 5, 2016
This is version 65 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.