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
Mus musculus (Mouse)
Status
Reviewed-Annotation score: -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

GO - Biological processi

Keywordsi

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

Enzyme and pathway databases

ReactomeiR-MMU-1268020 Mitochondrial protein import
R-MMU-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
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaMyomorphaMuroideaMuridaeMurinaeMusMus
Proteomesi
  • UP000000589 Componenti: Chromosome 19

Organism-specific databases

MGIiMGI:1096879 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

Pathology & Biotechi

Disruption phenotypei

Loss of cell division and lethal in fibroblasts.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Transit peptidei1 – 40MitochondrionBy similarityAdd BLAST40
ChainiPRO_000001013241 – 207Frataxin intermediate formAdd BLAST167
ChainiPRO_000039939078 – 207Frataxin 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

EPDiO35943
PaxDbiO35943
PeptideAtlasiO35943
PRIDEiO35943

PTM databases

iPTMnetiO35943
PhosphoSitePlusiO35943

Expressioni

Tissue specificityi

Heart, liver, skeletal muscle, kidney, spleen and thymus. Weakly expressed in the brain and lung.1 Publication

Developmental stagei

Expression in the ventricular zone which corresponds to dividing neuronal precursors begins at day 12.5, increases during embryonic development and persists at postnatal day 7 (P7) in the ependymal layer, which is the remenant of the ventricular zone. Weak expression seen in the spinal cord and medulla oblongata, starting at embryonic day 14.5 (E14.5) and expression also observed in dorsal root ganglia, starting at E14.5. At P14, expression in the dorsal root ganglia is restricted to the cortical region where the sensory neuron cell bodies are located. In non-neural tissues strong expression seen in the developing liver from E10.5. Expression detected in the heart and in the cortex of the developing kidney at E12.5 and later. Very high expression observed in the brown adipose tissue. Expression seen in small islands around the neck and back at E14.5, then in large masses at E16.5 and E18.5 and at P14 is absent in brown adipose tissue. Expression also seen in the thymus and developing gut at E14.5 and until postnatal life. At P14, expression in thymus is restricted to the proliferating cells in the cortical zone and is also prominent in the spleen. Found in the lung at E14.5.1 Publication

Gene expression databases

BgeeiENSMUSG00000059363 Expressed in 99 organ(s), highest expression level in quadriceps femoris
CleanExiMM_FXN
ExpressionAtlasiO35943 baseline and differential
GenevisibleiO35943 MM

Interactioni

Subunit structurei

Monomer (probable predominant form). Oligomer (PubMed:11823441). 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 similarity1 Publication

GO - Molecular functioni

Protein-protein interaction databases

IntActiO35943, 2 interactors
MINTiO35943
STRINGi10090.ENSMUSP00000080081

Structurei

3D structure databases

ProteinModelPortaliO35943
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
InParanoidiO35943
KOiK19054
OMAiKQSVCLM
OrthoDBiEOG091G0UR5
PhylomeDBiO35943
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

Sequence (1+)i

Sequence statusi: Complete.

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

This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All

O35943-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MWAFGGRAAV GLLPRTASRA SAWVGNPRWR EPIVTCGRRG LHVTVNAGAT
60 70 80 90 100
RHAHLNLHYL QILNIKKQSV CVVHLRNLGT LDNPSSLDET AYERLAEETL
110 120 130 140 150
DSLAEFFEDL ADKPYTLEDY DVSFGDGVLT IKLGGDLGTY VINKQTPNKQ
160 170 180 190 200
IWLSSPSSGP KRYDWTGKNW VYSHDGVSLH ELLARELTKA LNTKLDLSSL

AYSGKGT
Length:207
Mass (Da):22,924
Last modified:January 1, 1998 - v1
Checksum:iC46FD21B44FB26A2
GO

Computationally mapped potential isoform sequencesi

There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
E9Q2P9E9Q2P9_MOUSE
Frataxin, mitochondrial
Fxn
145Annotation score:

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U95736 mRNA Translation: AAB67778.1
CCDSiCCDS29711.1
RefSeqiNP_032070.1, NM_008044.2
UniGeneiMm.7319

Genome annotation databases

EnsembliENSMUST00000081333; ENSMUSP00000080081; ENSMUSG00000059363
GeneIDi14297
KEGGimmu:14297
UCSCiuc008hao.1 mouse

Similar proteinsi

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
U95736 mRNA Translation: AAB67778.1
CCDSiCCDS29711.1
RefSeqiNP_032070.1, NM_008044.2
UniGeneiMm.7319

3D structure databases

ProteinModelPortaliO35943
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiO35943, 2 interactors
MINTiO35943
STRINGi10090.ENSMUSP00000080081

PTM databases

iPTMnetiO35943
PhosphoSitePlusiO35943

Proteomic databases

EPDiO35943
PaxDbiO35943
PeptideAtlasiO35943
PRIDEiO35943

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENSMUST00000081333; ENSMUSP00000080081; ENSMUSG00000059363
GeneIDi14297
KEGGimmu:14297
UCSCiuc008hao.1 mouse

Organism-specific databases

CTDi2395
MGIiMGI:1096879 Fxn

Phylogenomic databases

eggNOGiKOG3413 Eukaryota
COG1965 LUCA
GeneTreeiENSGT00390000005811
HOGENOMiHOG000190729
HOVERGENiHBG005745
InParanoidiO35943
KOiK19054
OMAiKQSVCLM
OrthoDBiEOG091G0UR5
PhylomeDBiO35943
TreeFamiTF318958

Enzyme and pathway databases

ReactomeiR-MMU-1268020 Mitochondrial protein import
R-MMU-1362409 Mitochondrial iron-sulfur cluster biogenesis

Miscellaneous databases

PROiPR:O35943
SOURCEiSearch...

Gene expression databases

BgeeiENSMUSG00000059363 Expressed in 99 organ(s), highest expression level in quadriceps femoris
CleanExiMM_FXN
ExpressionAtlasiO35943 baseline and differential
GenevisibleiO35943 MM

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
ProtoNetiSearch...

Entry informationi

Entry nameiFRDA_MOUSE
AccessioniPrimary (citable) accession number: O35943
Entry historyiIntegrated into UniProtKB/Swiss-Prot: July 15, 1999
Last sequence update: January 1, 1998
Last modified: November 7, 2018
This is version 140 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

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again