Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.
Protein

Frataxin, mitochondrial

Gene

FXN

Organism
Bos taurus (Bovine)
Status
Reviewed-Annotation score: -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

Keywordsi

Molecular functionOxidoreductase
Biological processHeme biosynthesis, Ion transport, Iron storage, Iron transport, Transport
LigandIron

Enzyme and pathway databases

ReactomeiR-BTA-1268020 Mitochondrial protein import
R-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

Organism-specific databases

VGNCiVGNC:29155 FXN

Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

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

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
InterProiView protein in InterPro
IPR017789 Frataxin
IPR002908 Frataxin/CyaY
IPR036524 Frataxin/CyaY_sf
IPR020895 Frataxin_CS
PANTHERiPTHR16821 PTHR16821, 1 hit
PfamiView protein in Pfam
PF01491 Frataxin_Cyay, 1 hit
PRINTSiPR00904 FRATAXIN
SMARTiView protein in SMART
SM01219 Frataxin_Cyay, 1 hit
TIGRFAMsiTIGR03421 FeS_CyaY, 1 hit
TIGR03422 mito_frataxin, 1 hit
PROSITEiView protein in PROSITE
PS01344 FRATAXIN_1, 1 hit
PS50810 FRATAXIN_2, 1 hit

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

Similar proteinsi

Entry informationi

Entry nameiFRDA_BOVIN
AccessioniPrimary (citable) accession number: Q05B87
Entry historyiIntegrated into UniProtKB/Swiss-Prot: May 29, 2007
Last sequence update: November 14, 2006
Last modified: April 25, 2018
This is version 72 of the entry and version 1 of the sequence. See complete history.
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health