Q8HXX9 (FRDA_MACFA) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended 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

Organism

Macaca fascicularis (Crab-eating macaque) (Cynomolgus monkey)

Taxonomic identifier

9541 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Cercopithecoidea › Cercopithecidae › Cercopithecinae › Macaca

Protein attributes

Sequence length	210 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence 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.

Ontologies

Keywords
Biological process	Heme biosynthesis Ion transport Iron storage Iron transport Transport
Cellular component	Cytoplasm Mitochondrion
Domain	Transit peptide
Ligand	Iron
Molecular function	Oxidoreductase
Gene Ontology (GO)
Biological_process	cellular iron ion homeostasis Inferred from electronic annotation. Source: UniProtKB-KW heme biosynthetic process Inferred from electronic annotation. Source: UniProtKB-KW ion transport Inferred from electronic annotation. Source: UniProtKB-KW iron-sulfur cluster assembly Inferred from electronic annotation. Source: InterPro
Cellular_component	mitochondrion Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular_function	ferric iron binding Inferred from electronic annotation. Source: InterPro ferroxidase activity Inferred from electronic annotation. Source: EC
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 41	41	Mitochondrion By similarity
	Chain	42 – 210	169	Frataxin intermediate form		PRO_0000010131
	Chain	81 – 210	130	Frataxin mature form By similarity		PRO_0000399389

Sequences

	Sequence		Length	Mass (Da)
	Q8HXX9 [UniParc]. Last modified March 1, 2003. Version 1. Checksum: C90A50A3F3E7D581	FASTA	210	23,133
	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

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.
3D structure databases
ProteinModelPortal	Q8HXX9.
SMR	Q8HXX9. Positions 90-210.
ModBase	Search...
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Phylogenomic databases
HOVERGEN	HBG005745.
Family and domain databases
Gene3D	3.30.920.10. 1 hit.
InterPro	IPR017789. Frataxin. IPR002908. Frataxin/CyaY. IPR020895. Frataxin_CS. [Graphical view]
PANTHER	PTHR16821. PTHR16821. 1 hit.
Pfam	PF01491. Frataxin_Cyay. 1 hit. [Graphical view]
PRINTS	PR00904. FRATAXIN.
SUPFAM	SSF55387. Frataxin_like. 1 hit.
TIGRFAMs	TIGR03421. FeS_CyaY. 1 hit. TIGR03422. mito_frataxin. 1 hit.
PROSITE	PS01344. FRATAXIN_1. 1 hit. PS50810. FRATAXIN_2. 1 hit. [Graphical view]
ProtoNet	Search...

Entry information

Entry name

FRDA_MACFA

Accession

Primary (citable) accession number: Q8HXX9

Entry history

Integrated into UniProtKB/Swiss-Prot:	April 12, 2005
Last sequence update:	March 1, 2003
Last modified:	March 6, 2013
This is version 49 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program