UniProt Accession

 FRDA_MOUSE; O35943 

Genbank Protein ID

 U95736 

Genbank Nucleotide ID

 AAB67778.1 

Protein Name

 Frataxin, mitochondrial 

Protein Synonyms/Alias

 Fxn; Frataxin intermediate form; Frataxin mature form 

Gene Name

 Fxn 

Gene Synonyms/Alias

 Frda 

Created Date

 July 27, 2013 

Organism

 Mus musculus (Mouse) 

NCBI Taxa ID

 10090 

Lysine Modification

Position	Peptide	Type	References
189	LLARELTKALNTKLD	acetylation	[1]
194	LTKALNTKLDLSSLA	acetylation	[1, 2]

Reference

 [1] Label-free quantitative proteomics of the lysine acetylome in mitochondria identifies substrates of SIRT3 in metabolic pathways.
 Rardin MJ, Newman JC, Held JM, Cusack MP, Sorensen DJ, Li B, Schilling B, Mooney SD, Kahn CR, Verdin E, Gibson BW.
 Proc Natl Acad Sci U S A. 2013 Apr 16;110(16):6601-6. [PMID: 23576753]
 [2] Calorie restriction and SIRT3 trigger global reprogramming of the mitochondrial protein acetylome.
 Hebert AS, Dittenhafer-Reed KE, Yu W, Bailey DJ, Selen ES, Boersma MD, Carson JJ, Tonelli M, Balloon AJ, Higbee AJ, Westphall MS, Pagliarini DJ, Prolla TA, Assadi-Porter F, Roy S, Denu JM, Coon JJ.
 Mol Cell. 2013 Jan 10;49(1):186-99. [PMID: 23201123] 

Functional Description

 Promotes the biosynthesis of heme and assembly and repair of iron-sulfur clusters by delivering Fe(2+) 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 Fe(2+) to Fe(3+); 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). 

Sequence Annotation

  

Keyword

 Complete proteome; Cytoplasm; Heme biosynthesis; Ion transport; Iron; Iron storage; Iron transport; Metal-binding; Mitochondrion; Oxidoreductase; Reference proteome; Transit peptide; Transport. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 207 AA 

Protein Sequence

Gene Ontology

 GO:0005829; C:cytosol; IEA:Compara.
 GO:0005739; C:mitochondrion; IDA:MGI.
 GO:0051537; F:2 iron, 2 sulfur cluster binding; IEA:Compara.
 GO:0008199; F:ferric iron binding; IEA:InterPro.
 GO:0008198; F:ferrous iron binding; IEA:Compara.
 GO:0004322; F:ferroxidase activity; IEA:EC.
 GO:0034986; F:iron chaperone activity; IEA:Compara.
 GO:0007628; P:adult walking behavior; IMP:MGI.
 GO:0009060; P:aerobic respiration; IMP:MGI.
 GO:0006879; P:cellular iron ion homeostasis; IMP:MGI.
 GO:0070301; P:cellular response to hydrogen peroxide; IEA:Compara.
 GO:0009792; P:embryo development ending in birth or egg hatching; IMP:MGI.
 GO:0006783; P:heme biosynthetic process; IEA:UniProtKB-KW.
 GO:0006811; P:ion transport; IEA:UniProtKB-KW.
 GO:0018283; P:iron incorporation into metallo-sulfur cluster; IEA:Compara.
 GO:0016226; P:iron-sulfur cluster assembly; IMP:MGI.
 GO:0007005; P:mitochondrion organization; IMP:MGI.
 GO:0043066; P:negative regulation of apoptotic process; IEA:Compara.
 GO:0040015; P:negative regulation of multicellular organism growth; IMP:MGI.
 GO:0046621; P:negative regulation of organ growth; IMP:MGI.
 GO:0090201; P:negative regulation of release of cytochrome c from mitochondria; IEA:Compara.
 GO:0006119; P:oxidative phosphorylation; IMP:MGI.
 GO:0045773; P:positive regulation of axon extension; IEA:Compara.
 GO:0008284; P:positive regulation of cell proliferation; IEA:Compara.
 GO:0051349; P:positive regulation of lyase activity; IEA:Compara.
 GO:0048554; P:positive regulation of metalloenzyme activity; IEA:Compara.
 GO:0051353; P:positive regulation of oxidoreductase activity; IEA:Compara.
 GO:0051347; P:positive regulation of transferase activity; IEA:Compara.
 GO:0019230; P:proprioception; IMP:MGI.
 GO:0016540; P:protein autoprocessing; IEA:Compara.
 GO:0010722; P:regulation of ferrochelatase activity; IEA:Compara.
 GO:0042493; P:response to drug; IEA:Compara.
 GO:0010039; P:response to iron ion; IEA:Compara.
 GO:0014070; P:response to organic cyclic compound; IEA:Compara. 

Interpro

 IPR017789; Frataxin.
 IPR002908; Frataxin/CyaY.
 IPR020895; Frataxin_CS. 

Pfam

 PF01491; Frataxin_Cyay 

SMART

  

PROSITE

 PS01344; FRATAXIN_1
 PS50810; FRATAXIN_2 

PRINTS

 PR00904; FRATAXIN.