Q05B87 (FRDA_BOVIN) 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:	FRDA

Organism

Bos taurus (Bovine) [Reference proteome]

Taxonomic identifier

9913 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Laurasiatheria › Cetartiodactyla › Ruminantia › Pecora › Bovidae › Bovinae › Bos

Protein attributes

Sequence length	217 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
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	adult walking behavior Inferred from electronic annotation. Source: Compara aerobic respiration Inferred from electronic annotation. Source: Compara cellular iron ion homeostasis Inferred from electronic annotation. Source: UniProtKB-KW cellular response to hydrogen peroxide Inferred from electronic annotation. Source: Compara embryo development ending in birth or egg hatching Inferred from electronic annotation. Source: Compara heme biosynthetic process Inferred from electronic annotation. Source: UniProtKB-KW ion transport Inferred from electronic annotation. Source: UniProtKB-KW iron incorporation into metallo-sulfur cluster Inferred from electronic annotation. Source: Compara mitochondrion organization Inferred from electronic annotation. Source: Compara negative regulation of apoptotic process Inferred from electronic annotation. Source: Compara negative regulation of multicellular organism growth Inferred from electronic annotation. Source: Compara negative regulation of organ growth Inferred from electronic annotation. Source: Compara negative regulation of release of cytochrome c from mitochondria Inferred from electronic annotation. Source: Compara oxidative phosphorylation Inferred from electronic annotation. Source: Compara positive regulation of cell growth Inferred from electronic annotation. Source: Compara positive regulation of cell proliferation Inferred from electronic annotation. Source: Compara positive regulation of lyase activity Inferred from electronic annotation. Source: Compara positive regulation of metalloenzyme activity Inferred from electronic annotation. Source: Compara positive regulation of oxidoreductase activity Inferred from electronic annotation. Source: Compara positive regulation of transferase activity Inferred from electronic annotation. Source: Compara proprioception Inferred from electronic annotation. Source: Compara protein autoprocessing Inferred from electronic annotation. Source: Compara regulation of ferrochelatase activity Inferred from electronic annotation. Source: Compara response to iron ion Inferred from electronic annotation. Source: Compara
Cellular_component	cytosol Inferred from electronic annotation. Source: Compara mitochondrion Inferred from electronic annotation. Source: UniProtKB-SubCell
Molecular_function	2 iron, 2 sulfur cluster binding Inferred from electronic annotation. Source: Compara ferric iron binding Inferred from electronic annotation. Source: InterPro ferrous iron binding Inferred from electronic annotation. Source: Compara ferroxidase activity Inferred from electronic annotation. Source: EC iron chaperone activity Inferred from electronic annotation. Source: Compara
Complete GO annotation...

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 42	42	Mitochondrion By similarity
	Chain	43 – 217	175	Frataxin intermediate form		PRO_0000289330
	Chain	82 – 217	136	Frataxin mature form By similarity		PRO_0000399387

Sequences

	Sequence		Length	Mass (Da)
	Q05B87 [UniParc]. Last modified November 14, 2006. Version 1. Checksum: AF8AB00650CD9ACC	FASTA	217	23,567
	10 20 30 40 50 60 MWTLGRRSVA SFLPRSALPG FAPTRAGAPR PAKDLSLSGL PGLRIGTAKA PARSQSSLSL 70 80 90 100 110 120 RCLNQTLDVK KQSVCWINLR TAGTLGDAGT LDDTTYERLA EETLDSLAEF FEDLADKPYT 130 140 150 160 170 180 FEDYDVSFGS GVLTVKLGGD LGTYVINKQT PNKQIWLSSP SSGPKRYDWT GRNWVYSHDG 190 200 210 VSLHELLATE LTQALKTKLD LSALAYSGKD TCCPAQC

References

[1]	NIH - Mammalian Gene Collection (MGC) project Submitted (AUG-2006) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Strain: Hereford. Tissue: Thalamus.

Cross-references

Sequence databases
EMBL GenBank DDBJ	BC122620 mRNA. Translation: AAI22621.1.
IPI	IPI00709291.
RefSeq	NP_001074196.1. NM_001080727.1.
UniGene	Bt.17029.
3D structure databases
ProteinModelPortal	Q05B87.
SMR	Q05B87. Positions 91-210.
ModBase	Search...
Protein-protein interaction databases
STRING	9913.ENSBTAP00000001725.
Proteomic databases
PRIDE	Q05B87.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENSBTAT00000001725; ENSBTAP00000001725; ENSBTAG00000001306.
GeneID	505694.
KEGG	bta:505694.
Organism-specific databases
CTD	2395.
Phylogenomic databases
eggNOG	COG1965.
GeneTree	ENSGT00390000005811.
HOGENOM	HOG000190729.
HOVERGEN	HBG005745.
InParanoid	Q05B87.
OMA	KQSVCLM.
OrthoDB	EOG4ZCT5S.
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...
Other
NextBio	20867270.

Entry information

Entry name

FRDA_BOVIN

Accession

Primary (citable) accession number: Q05B87

Entry history

Integrated into UniProtKB/Swiss-Prot:	May 29, 2007
Last sequence update:	November 14, 2006
Last modified:	April 3, 2013
This is version 44 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program