UniProtKB - O35943 (FRDA_MOUSE)
Frataxin, mitochondrial
Fxn
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 PublicationCatalytic activityi
- EC:1.16.3.1
GO - Molecular functioni
- 2 iron, 2 sulfur cluster binding Source: MGI
- enzyme binding Source: MGI
- ferric iron binding Source: MGI
- ferrous iron binding Source: MGI
- ferroxidase activity Source: MGI
- iron chaperone activity Source: MGI
GO - Biological processi
- adult walking behavior Source: MGI
- aerobic respiration Source: MGI
- cellular iron ion homeostasis Source: MGI
- cellular response to hydrogen peroxide Source: MGI
- embryo development ending in birth or egg hatching Source: MGI
- heme biosynthetic process Source: UniProtKB-KW
- ion transport Source: UniProtKB-KW
- iron incorporation into metallo-sulfur cluster Source: MGI
- iron-sulfur cluster assembly Source: MGI
- mitochondrion organization Source: MGI
- negative regulation of apoptotic process Source: MGI
- negative regulation of multicellular organism growth Source: MGI
- negative regulation of organ growth Source: MGI
- negative regulation of release of cytochrome c from mitochondria Source: MGI
- oxidative phosphorylation Source: MGI
- positive regulation of aconitate hydratase activity Source: MGI
- positive regulation of axon extension Source: MGI
- positive regulation of catalytic activity Source: MGI
- positive regulation of cell growth Source: MGI
- positive regulation of cell population proliferation Source: MGI
- positive regulation of lyase activity Source: MGI
- positive regulation of succinate dehydrogenase activity Source: MGI
- proprioception Source: MGI
- protein autoprocessing Source: MGI
- regulation of ferrochelatase activity Source: MGI
- response to iron ion Source: MGI
Keywordsi
Molecular function | Oxidoreductase |
Biological process | Heme biosynthesis, Ion transport, Iron storage, Iron transport, Transport |
Ligand | Iron, Metal-binding |
Enzyme and pathway databases
Reactomei | R-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 |
Organismi | Mus musculus (Mouse) |
Taxonomic identifieri | 10090 [NCBI] |
Taxonomic lineagei | Eukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Glires › Rodentia › Myomorpha › Muroidea › Muridae › Murinae › Mus › Mus |
Proteomesi |
|
Organism-specific databases
MGIi | MGI:1096879, Fxn |
VEuPathDBi | HostDB:ENSMUSG00000059363 |
Subcellular locationi
Mitochondrion
- Mitochondrion 2 Publications
Cytoplasm and Cytosol
- cytosol By similarity
Note: PubMed:17597094 describes localization exclusively in mitochondria.
Cytosol
- cytosol Source: MGI
- iron-sulfur cluster assembly complex Source: ComplexPortal
- L-cysteine desulfurase complex Source: MGI
Mitochondrion
- mitochondrion Source: MGI
Keywords - Cellular componenti
Cytoplasm, MitochondrionPathology & Biotechi
Disruption phenotypei
PTM / Processingi
Molecule processing
Feature key | Position(s) | DescriptionActions | Graphical view | Length |
---|---|---|---|---|
Transit peptidei | 1 – 40 | MitochondrionBy similarityAdd BLAST | 40 | |
ChainiPRO_0000010132 | 41 – 207 | Frataxin intermediate formAdd BLAST | 167 | |
ChainiPRO_0000399390 | 78 – 207 | Frataxin mature formBy similarityAdd BLAST | 130 |
Post-translational modificationi
Proteomic databases
EPDi | O35943 |
jPOSTi | O35943 |
PaxDbi | O35943 |
PeptideAtlasi | O35943 |
PRIDEi | O35943 |
ProteomicsDBi | 267517 |
PTM databases
iPTMneti | O35943 |
PhosphoSitePlusi | O35943 |
Expressioni
Tissue specificityi
Developmental stagei
Gene expression databases
Bgeei | ENSMUSG00000059363, Expressed in adult mammalian kidney and 137 other tissues |
ExpressionAtlasi | O35943, baseline and differential |
Genevisiblei | O35943, 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 PublicationGO - Molecular functioni
- enzyme binding Source: MGI
Protein-protein interaction databases
BioGRIDi | 199741, 5 interactors |
ComplexPortali | CPX-5823, Mitochondrial NIAUFX iron-sulfur cluster assembly complex |
STRINGi | 10090.ENSMUSP00000080081 |
Miscellaneous databases
RNActi | O35943, protein |
Family & Domainsi
Sequence similaritiesi
Keywords - Domaini
Transit peptidePhylogenomic databases
eggNOGi | KOG3413, Eukaryota |
GeneTreei | ENSGT00390000005811 |
HOGENOMi | CLU_080880_1_0_1 |
InParanoidi | O35943 |
OMAi | CWINLRT |
OrthoDBi | 1372185at2759 |
PhylomeDBi | O35943 |
TreeFami | TF318958 |
Family and domain databases
Gene3Di | 3.30.920.10, 1 hit |
InterProi | View protein in InterPro IPR017789, Frataxin IPR002908, Frataxin/CyaY IPR036524, Frataxin/CyaY_sf IPR020895, Frataxin_CS |
PANTHERi | PTHR16821, PTHR16821, 1 hit |
Pfami | View protein in Pfam PF01491, Frataxin_Cyay, 1 hit |
PRINTSi | PR00904, FRATAXIN |
SMARTi | View protein in SMART SM01219, Frataxin_Cyay, 1 hit |
SUPFAMi | SSF55387, SSF55387, 1 hit |
TIGRFAMsi | TIGR03421, FeS_CyaY, 1 hit TIGR03422, mito_frataxin, 1 hit |
PROSITEi | View protein in PROSITE PS01344, FRATAXIN_1, 1 hit PS50810, FRATAXIN_2, 1 hit |
(1+)i Sequence
Sequence statusi: Complete.
: The displayed sequence is further processed into a mature form. Sequence processingi
This entry has 1 described isoform and 1 potential isoform that is computationally mapped.Show allAlign All
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
Computationally mapped potential isoform sequencesi
There is 1 potential isoform mapped to this entry.BLASTAlignShow allAdd to basketE9Q2P9 | E9Q2P9_MOUSE | Frataxin, mitochondrial | Fxn | 145 | Annotation score: |
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | U95736 mRNA Translation: AAB67778.1 |
CCDSi | CCDS29711.1 |
RefSeqi | NP_032070.1, NM_008044.2 |
Genome annotation databases
Ensembli | ENSMUST00000081333; ENSMUSP00000080081; ENSMUSG00000059363 |
GeneIDi | 14297 |
KEGGi | mmu:14297 |
UCSCi | uc008hao.1, mouse |
Similar proteinsi
Cross-referencesi
Sequence databases
Select the link destinations: EMBLi GenBanki DDBJi Links Updated | U95736 mRNA Translation: AAB67778.1 |
CCDSi | CCDS29711.1 |
RefSeqi | NP_032070.1, NM_008044.2 |
3D structure databases
AlphaFoldDBi | O35943 |
SMRi | O35943 |
ModBasei | Search... |
Protein-protein interaction databases
BioGRIDi | 199741, 5 interactors |
ComplexPortali | CPX-5823, Mitochondrial NIAUFX iron-sulfur cluster assembly complex |
STRINGi | 10090.ENSMUSP00000080081 |
PTM databases
iPTMneti | O35943 |
PhosphoSitePlusi | O35943 |
Proteomic databases
EPDi | O35943 |
jPOSTi | O35943 |
PaxDbi | O35943 |
PeptideAtlasi | O35943 |
PRIDEi | O35943 |
ProteomicsDBi | 267517 |
Protocols and materials databases
ABCDi | O35943, 1 sequenced antibody |
Antibodypediai | 26793, 619 antibodies from 35 providers |
DNASUi | 14297 |
Genome annotation databases
Ensembli | ENSMUST00000081333; ENSMUSP00000080081; ENSMUSG00000059363 |
GeneIDi | 14297 |
KEGGi | mmu:14297 |
UCSCi | uc008hao.1, mouse |
Organism-specific databases
CTDi | 2395 |
MGIi | MGI:1096879, Fxn |
VEuPathDBi | HostDB:ENSMUSG00000059363 |
Phylogenomic databases
eggNOGi | KOG3413, Eukaryota |
GeneTreei | ENSGT00390000005811 |
HOGENOMi | CLU_080880_1_0_1 |
InParanoidi | O35943 |
OMAi | CWINLRT |
OrthoDBi | 1372185at2759 |
PhylomeDBi | O35943 |
TreeFami | TF318958 |
Enzyme and pathway databases
Reactomei | R-MMU-1268020, Mitochondrial protein import R-MMU-1362409, Mitochondrial iron-sulfur cluster biogenesis |
Miscellaneous databases
BioGRID-ORCSi | 14297, 26 hits in 72 CRISPR screens |
ChiTaRSi | Fxn, mouse |
PROi | PR:O35943 |
RNActi | O35943, protein |
SOURCEi | Search... |
Gene expression databases
Bgeei | ENSMUSG00000059363, Expressed in adult mammalian kidney and 137 other tissues |
ExpressionAtlasi | O35943, baseline and differential |
Genevisiblei | O35943, MM |
Family and domain databases
Gene3Di | 3.30.920.10, 1 hit |
InterProi | View protein in InterPro IPR017789, Frataxin IPR002908, Frataxin/CyaY IPR036524, Frataxin/CyaY_sf IPR020895, Frataxin_CS |
PANTHERi | PTHR16821, PTHR16821, 1 hit |
Pfami | View protein in Pfam PF01491, Frataxin_Cyay, 1 hit |
PRINTSi | PR00904, FRATAXIN |
SMARTi | View protein in SMART SM01219, Frataxin_Cyay, 1 hit |
SUPFAMi | SSF55387, SSF55387, 1 hit |
TIGRFAMsi | TIGR03421, FeS_CyaY, 1 hit TIGR03422, mito_frataxin, 1 hit |
PROSITEi | View protein in PROSITE PS01344, FRATAXIN_1, 1 hit PS50810, FRATAXIN_2, 1 hit |
MobiDBi | Search... |
Entry informationi
Entry namei | FRDA_MOUSE | |
Accessioni | O35943Primary (citable) accession number: O35943 | |
Entry historyi | Integrated into UniProtKB/Swiss-Prot: | July 15, 1999 |
Last sequence update: | January 1, 1998 | |
Last modified: | May 25, 2022 | |
This is version 158 of the entry and version 1 of the sequence. See complete history. | ||
Entry statusi | Reviewed (UniProtKB/Swiss-Prot) | |
Annotation program | Chordata Protein Annotation Program |
Miscellaneousi
Keywords - Technical termi
Reference proteomeDocuments
- MGD cross-references
Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot - SIMILARITY comments
Index of protein domains and families