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Q8HXX9 (FRDA_MACFA) Reviewed, UniProtKB/Swiss-Prot

Last modified April 16, 2014. Version 53. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (1) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Frataxin, mitochondrial

Short name=Fxn
EC=1.16.3.1

Cleaved into the following 2 chains:

  1. Frataxin intermediate form
  2. Frataxin mature form
Gene names
Name:FXN
Synonyms:FRDA1
ORF Names:QnpA-13971
OrganismMacaca fascicularis (Crab-eating macaque) (Cynomolgus monkey)
Taxonomic identifier9541 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniCercopithecidaeCercopithecinaeMacaca

Protein attributes

Sequence length210 AA.
Sequence statusComplete.
Sequence processingThe displayed sequence is further processed into a mature form.
Protein existenceEvidence at transcript level

General annotation (Comments)

Function

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.

Catalytic activity

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

Subunit structure

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.

Subcellular location

Cytoplasm By similarity. Mitochondrion By similarity.

Post-translational modification

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.

Sequence similarities

Belongs to the frataxin family.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Transit peptide1 – 4141Mitochondrion By similarity
Chain42 – 210169Frataxin intermediate form
PRO_0000010131
Chain81 – 210130Frataxin mature form By similarity
PRO_0000399389

Sequences

Sequence LengthMass (Da)Tools
Q8HXX9 [UniParc].

Last modified March 1, 2003. Version 1.
Checksum: C90A50A3F3E7D581

FASTA21023,133
        10         20         30         40         50         60 
MWTFGRRAVA GLLASPSPAQ AQTLTRAPRL AELAQLCSRR GLRTGINATR TTHHTSSNLR 

        70         80         90        100        110        120 
GLNQIRNVKR QSVYLMNLRK SGTLGHPGSL DDTTYERLAE ETLDSLAEFF EDLADKPYTF 

       130        140        150        160        170        180 
EDYDVSFGSG VLTVKLGGDL GTYVINKQTP NKQIWLSSPS SGPKRYDRTG KNWVYSHDGV 

       190        200        210 
SLHELLGAEL TKALKTKLDL SSLAYSGKDA 

« Hide

References

[1]"Isolation and characterization of cDNA for macaque neurological disease genes."
Kusuda J., Osada N., Hida M., Sugano S., Hashimoto K.
Submitted (APR-2002) to the EMBL/GenBank/DDBJ databases
Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
Tissue: Parietal cortex.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
AB083310 mRNA. Translation: BAC20589.1.
RefSeqNP_001271967.1. NM_001285038.1.
UniGeneMfa.6270.

3D structure databases

ProteinModelPortalQ8HXX9.
SMRQ8HXX9. Positions 90-210.
ModBaseSearch...
MobiDBSearch...

Protocols and materials databases

StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

GeneID102120656.
KEGGmcf:102120656.

Organism-specific databases

CTD2395.

Phylogenomic databases

HOVERGENHBG005745.

Family and domain databases

Gene3D3.30.920.10. 1 hit.
InterProIPR017789. Frataxin.
IPR002908. Frataxin/CyaY.
IPR020895. Frataxin_CS.
[Graphical view]
PANTHERPTHR16821. PTHR16821. 1 hit.
PfamPF01491. Frataxin_Cyay. 1 hit.
[Graphical view]
PRINTSPR00904. FRATAXIN.
SUPFAMSSF55387. SSF55387. 1 hit.
TIGRFAMsTIGR03421. FeS_CyaY. 1 hit.
TIGR03422. mito_frataxin. 1 hit.
PROSITEPS01344. FRATAXIN_1. 1 hit.
PS50810. FRATAXIN_2. 1 hit.
[Graphical view]
ProtoNetSearch...

Entry information

Entry nameFRDA_MACFA
AccessionPrimary (citable) accession number: Q8HXX9
Entry history
Integrated into UniProtKB/Swiss-Prot: April 12, 2005
Last sequence update: March 1, 2003
Last modified: April 16, 2014
This is version 53 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Relevant documents

SIMILARITY comments

Index of protein domains and families