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Protein

Frataxin, mitochondrial

Gene

frh-1

Organism
Caenorhabditis elegans
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at transcript leveli

Functioni

Promotes the biosynthesis of heme as well as the 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+. May be able to store large amounts of the metal in the form of a ferrihydrite mineral by oligomerization.1 Publication

Catalytic activityi

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

GO - Molecular functioni

  1. ferric iron binding Source: InterPro
  2. ferroxidase activity Source: UniProtKB-EC

GO - Biological processi

  1. cellular iron ion homeostasis Source: UniProtKB-KW
  2. determination of adult lifespan Source: WormBase
  3. embryo development Source: WormBase
  4. growth Source: WormBase
  5. heme biosynthetic process Source: UniProtKB-KW
  6. ion transport Source: UniProtKB-KW
  7. iron-sulfur cluster assembly Source: WormBase
  8. nematode larval development Source: WormBase
  9. response to hydrogen peroxide Source: WormBase
  10. response to superoxide Source: WormBase
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

ReactomeiREACT_183224. Mitochondrial iron-sulfur cluster biogenesis.
REACT_183227. Mitochondrial protein import.

Names & Taxonomyi

Protein namesi
Recommended name:
Frataxin, mitochondrial (EC:1.16.3.1)
Short name:
Fxn
Gene namesi
Name:frh-1
ORF Names:F59G1.7
OrganismiCaenorhabditis elegans
Taxonomic identifieri6239 [NCBI]
Taxonomic lineageiEukaryotaMetazoaEcdysozoaNematodaChromadoreaRhabditidaRhabditoideaRhabditidaePeloderinaeCaenorhabditis
ProteomesiUP000001940: Chromosome II

Organism-specific databases

WormBaseiF59G1.7; CE19476; WBGene00001486; frh-1.

Subcellular locationi

Mitochondrion By similarity

GO - Cellular componenti

  1. mitochondrion Source: WormBase
Complete GO annotation...

Keywords - Cellular componenti

Mitochondrion

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini? – 136Frataxin, mitochondrialPRO_0000193925
Transit peptidei1 – ?MitochondrionSequence Analysis

Proteomic databases

PaxDbiQ9TY03.

Interactioni

Subunit structurei

Monomer. Oligomer (By similarity).By similarity

Protein-protein interaction databases

IntActiQ9TY03. 1 interaction.
STRINGi6239.F59G1.7.2.

Structurei

3D structure databases

ProteinModelPortaliQ9TY03.
SMRiQ9TY03. Positions 22-133.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Sequence similaritiesi

Belongs to the frataxin family.Curated

Keywords - Domaini

Transit peptide

Phylogenomic databases

eggNOGiCOG1965.
GeneTreeiENSGT00390000005811.
HOGENOMiHOG000190729.
InParanoidiQ9TY03.
OMAiYEVEYHS.
OrthoDBiEOG7HHWVD.
PhylomeDBiQ9TY03.

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.
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.

Q9TY03-1 [UniParc]FASTAAdd to Basket

« Hide

        10         20         30         40         50
MLSTILRNNF VRRSFSSRIF SQNEYETAAD STLERLSDYF DQIADSFPVS
60 70 80 90 100
EQFDVSHAMG VLTVNVSKSV GTYVINKQSP NKQIWLSSPM SGPKRYDLEE
110 120 130
EGKWTYAHDG EQLDSLLNRE FRKILADDRI DFSRHV
Length:136
Mass (Da):15,718
Last modified:May 1, 2000 - v1
Checksum:i0ACB254346E38272
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY048153 mRNA. Translation: AAL05950.1.
FO081244 Genomic DNA. Translation: CCD70156.1.
PIRiT34316.
RefSeqiNP_495183.1. NM_062782.3.
UniGeneiCel.20708.

Genome annotation databases

EnsemblMetazoaiF59G1.7; F59G1.7; WBGene00001486.
GeneIDi174002.
KEGGicel:CELE_F59G1.7.
UCSCiF59G1.7.1. c. elegans.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY048153 mRNA. Translation: AAL05950.1.
FO081244 Genomic DNA. Translation: CCD70156.1.
PIRiT34316.
RefSeqiNP_495183.1. NM_062782.3.
UniGeneiCel.20708.

3D structure databases

ProteinModelPortaliQ9TY03.
SMRiQ9TY03. Positions 22-133.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

IntActiQ9TY03. 1 interaction.
STRINGi6239.F59G1.7.2.

Proteomic databases

PaxDbiQ9TY03.

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsemblMetazoaiF59G1.7; F59G1.7; WBGene00001486.
GeneIDi174002.
KEGGicel:CELE_F59G1.7.
UCSCiF59G1.7.1. c. elegans.

Organism-specific databases

CTDi174002.
WormBaseiF59G1.7; CE19476; WBGene00001486; frh-1.

Phylogenomic databases

eggNOGiCOG1965.
GeneTreeiENSGT00390000005811.
HOGENOMiHOG000190729.
InParanoidiQ9TY03.
OMAiYEVEYHS.
OrthoDBiEOG7HHWVD.
PhylomeDBiQ9TY03.

Enzyme and pathway databases

ReactomeiREACT_183224. Mitochondrial iron-sulfur cluster biogenesis.
REACT_183227. Mitochondrial protein import.

Miscellaneous databases

NextBioi882063.
PROiQ9TY03.

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.
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...

Publicationsi

« Hide 'large scale' publications
  1. "Characterization of the Caenorhabditis elegans ortholog of the frataxin."
    Vazquez-Manrique R.P., Baylis H.A., Palau F.
    Submitted (JUL-2001) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Strain: Bristol N2.
  2. "Genome sequence of the nematode C. elegans: a platform for investigating biology."
    The C. elegans sequencing consortium
    Science 282:2012-2018(1998) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    Strain: Bristol N2.
  3. "Reduction of Caenorhabditis elegans frataxin increases sensitivity to oxidative stress, reduces lifespan, and causes lethality in a mitochondrial complex II mutant."
    Vazquez-Manrique R.P., Gonzalez-Cabo P., Ros S., Aziz H., Baylis H.A., Palau F.
    FASEB J. 20:172-174(2006) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION.

Entry informationi

Entry nameiFRDA_CAEEL
AccessioniPrimary (citable) accession number: Q9TY03
Entry historyi
Integrated into UniProtKB/Swiss-Prot: December 1, 2000
Last sequence update: May 1, 2000
Last modified: January 7, 2015
This is version 86 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programCaenorhabditis annotation project

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Caenorhabditis elegans
    Caenorhabditis elegans: entries, gene names and cross-references to WormBase
  2. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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 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.