UniProt Accession

 FRDA_RAT; D3ZYW7 

Genbank Protein ID

 AABR03005338; AABR03006217; AABR03009513 

Genbank Nucleotide ID

  

Protein Name

 Frataxin, mitochondrial 

Protein Synonyms/Alias

 Fxn; Frataxin intermediate form; Frataxin mature form 

Gene Name

 Fxn 

Gene Synonyms/Alias

  

Created Date

 July 27, 2013 

Organism

 Rattus norvegicus (Rat) 

NCBI Taxa ID

 10116 

Lysine Modification

Position	Peptide	Type	References
169	KRYDWTGKNWVYSHD	acetylation	[1]

Reference

 [1] Proteomic analysis of lysine acetylation sites in rat tissues reveals organ specificity and subcellular patterns.
 Lundby A, Lage K, Weinert BT, Bekker-Jensen DB, Secher A, Skovgaard T, Kelstrup CD, Dmytriyev A, Choudhary C, Lundby C, Olsen JV.
 Cell Rep. 2012 Aug 30;2(2):419-31. [PMID: 22902405] 

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; Mitochondrion; Oxidoreductase; Reference proteome; Transit peptide; Transport. 

Sequence Source

 UniProt (SWISSPROT/TrEMBL); GenBank; EMBL 

Protein Length

 208 AA 

Protein Sequence

Gene Ontology

 GO:0005829; C:cytosol; IEA:Compara.
 GO:0005739; C:mitochondrion; IDA:RGD.
 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:0016530; F:metallochaperone activity; NAS:RGD.
 GO:0007628; P:adult walking behavior; IEA:Compara.
 GO:0009060; P:aerobic respiration; IEA:Compara.
 GO:0006879; P:cellular iron ion homeostasis; NAS:RGD.
 GO:0070301; P:cellular response to hydrogen peroxide; IEA:Compara.
 GO:0009792; P:embryo development ending in birth or egg hatching; IEA:Compara.
 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:0007005; P:mitochondrion organization; IEA:Compara.
 GO:0043066; P:negative regulation of apoptotic process; IEA:Compara.
 GO:0040015; P:negative regulation of multicellular organism growth; IEA:Compara.
 GO:0046621; P:negative regulation of organ growth; IEA:Compara.
 GO:0090201; P:negative regulation of release of cytochrome c from mitochondria; IEA:Compara.
 GO:0006119; P:oxidative phosphorylation; IEA:Compara.
 GO:0045773; P:positive regulation of axon extension; IMP:RGD.
 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; IEA:Compara.
 GO:0016540; P:protein autoprocessing; IEA:Compara.
 GO:0010722; P:regulation of ferrochelatase activity; IEA:Compara.
 GO:0042493; P:response to drug; IEP:RGD.
 GO:0010039; P:response to iron ion; IEA:Compara.
 GO:0014070; P:response to organic cyclic compound; IEP:RGD. 

Interpro

 IPR017789; Frataxin.
 IPR002908; Frataxin/CyaY. 

Pfam

 PF01491; Frataxin_Cyay 

SMART

  

PROSITE

 PS50810; FRATAXIN_2 

PRINTS

 PR00904; FRATAXIN.